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Surgery_Schwartz_10002 | Surgery_Schwartz | reported (and success-ful) splenectomy for a patient with idiopathic thrombocytopenia purpura.2As surgeons’ experience with the procedure grew, the associated morbidity and mortality decreased. By 1920, the Mayo Clinic reported that splenectomy had a reduced mortality rate of 11%.1O’Donnell in 1929 was the first to describe fatal post-splenectomy sepsis in a child who had undergone the sur-gery for hemolytic anemia.3 It took Springer’s 1973 review of almost 2800 postsplenectomy patients and the 2.5% inci-dence of sepsis-induced mortality (vs. 0.01% in the general population) to reorient surgeons to more conservative splenic procedures.2,3The advent of minimally invasive surgery and laparo-scopic splenectomy in the early 1990s represented a clear advance, benefitting the patient through this evolution of sur-gical technique. Most large series of laparoscopic splenectomy for benign and malignant indication now report a mortality rate of <1%.12,13 As even more contemporary research | Surgery_Schwartz. reported (and success-ful) splenectomy for a patient with idiopathic thrombocytopenia purpura.2As surgeons’ experience with the procedure grew, the associated morbidity and mortality decreased. By 1920, the Mayo Clinic reported that splenectomy had a reduced mortality rate of 11%.1O’Donnell in 1929 was the first to describe fatal post-splenectomy sepsis in a child who had undergone the sur-gery for hemolytic anemia.3 It took Springer’s 1973 review of almost 2800 postsplenectomy patients and the 2.5% inci-dence of sepsis-induced mortality (vs. 0.01% in the general population) to reorient surgeons to more conservative splenic procedures.2,3The advent of minimally invasive surgery and laparo-scopic splenectomy in the early 1990s represented a clear advance, benefitting the patient through this evolution of sur-gical technique. Most large series of laparoscopic splenectomy for benign and malignant indication now report a mortality rate of <1%.12,13 As even more contemporary research |
Surgery_Schwartz_10003 | Surgery_Schwartz | this evolution of sur-gical technique. Most large series of laparoscopic splenectomy for benign and malignant indication now report a mortality rate of <1%.12,13 As even more contemporary research reveals the spleen to play a central role in immune, metabolic, and endocrine function, it follows that the surgeon’s role going forward will be to preserve this organ and its functions when-ever possible.EMBRYOLOGY AND ANATOMYConsisting of an encapsulated mass of vascular and lymphoid tissue, the spleen is the largest reticuloendothelial organ in the body. Arising from the primitive mesoderm as an outgrowth of the left side of the dorsal mesogastrium, by the fifth week of gestation, the spleen is evident in an embryo 8 mm long.Development begins through the formation of the splanch-nic mesodermal plate, derived from the mesoderm, at embryonic day 12. The embryonic spleen is first colonized by erythroid and myeloid progenitor cells at 2 weeks of gestation. Follow-ing soon thereafter, the | Surgery_Schwartz. this evolution of sur-gical technique. Most large series of laparoscopic splenectomy for benign and malignant indication now report a mortality rate of <1%.12,13 As even more contemporary research reveals the spleen to play a central role in immune, metabolic, and endocrine function, it follows that the surgeon’s role going forward will be to preserve this organ and its functions when-ever possible.EMBRYOLOGY AND ANATOMYConsisting of an encapsulated mass of vascular and lymphoid tissue, the spleen is the largest reticuloendothelial organ in the body. Arising from the primitive mesoderm as an outgrowth of the left side of the dorsal mesogastrium, by the fifth week of gestation, the spleen is evident in an embryo 8 mm long.Development begins through the formation of the splanch-nic mesodermal plate, derived from the mesoderm, at embryonic day 12. The embryonic spleen is first colonized by erythroid and myeloid progenitor cells at 2 weeks of gestation. Follow-ing soon thereafter, the |
Surgery_Schwartz_10004 | Surgery_Schwartz | plate, derived from the mesoderm, at embryonic day 12. The embryonic spleen is first colonized by erythroid and myeloid progenitor cells at 2 weeks of gestation. Follow-ing soon thereafter, the hematopoietic stem cells take up resi-dence in the forming spleen.14 The spleen assumes an important hematopoietic role until the fifth month of gestation. After birth, splenic erythropoietic function may persist in some hematologic disorders.15Key Points1 The human spleen plays a key immunologic role in defense against a number of organisms, particularly encapsulated bacteria.2 The spleen can cause significant morbidity and/or hema-tologic disturbance if it becomes hyperfunctioning (hyper-splenism) or hypertrophied (splenomegaly).3 There is a broad spectrum of nontraumatic diseases for which elective splenectomy can be curative or palliative. They can be broadly categorized as red blood cell disor-ders and hemoglobinopathies, white blood cell disorders, platelet disorders, bone marrow | Surgery_Schwartz. plate, derived from the mesoderm, at embryonic day 12. The embryonic spleen is first colonized by erythroid and myeloid progenitor cells at 2 weeks of gestation. Follow-ing soon thereafter, the hematopoietic stem cells take up resi-dence in the forming spleen.14 The spleen assumes an important hematopoietic role until the fifth month of gestation. After birth, splenic erythropoietic function may persist in some hematologic disorders.15Key Points1 The human spleen plays a key immunologic role in defense against a number of organisms, particularly encapsulated bacteria.2 The spleen can cause significant morbidity and/or hema-tologic disturbance if it becomes hyperfunctioning (hyper-splenism) or hypertrophied (splenomegaly).3 There is a broad spectrum of nontraumatic diseases for which elective splenectomy can be curative or palliative. They can be broadly categorized as red blood cell disor-ders and hemoglobinopathies, white blood cell disorders, platelet disorders, bone marrow |
Surgery_Schwartz_10005 | Surgery_Schwartz | elective splenectomy can be curative or palliative. They can be broadly categorized as red blood cell disor-ders and hemoglobinopathies, white blood cell disorders, platelet disorders, bone marrow disorders, infections and abscesses, cysts and tumors, storage diseases and infiltra-tive disorders, and miscellaneous conditions.4 Inadvertent intraoperative splenic injury is a scenario for which every abdominal surgeon should be prepared. Avail-ability of a predetermined algorithm, with emphasis on the patient’s condition, facilitates intraoperative decision making.5 Partial splenectomy may be a suitable alternative to total splenectomy for certain conditions of hypersplen-ism or splenomegaly, particularly in children in whom preservation of splenic immunologic function is especially important.6 Preoperative splenic artery embolization for elective sple-nectomy has benefits and disadvantages. It may be most suitable in cases of enlarged spleen. Conclusive evidence is lacking.7 Vaccination | Surgery_Schwartz. elective splenectomy can be curative or palliative. They can be broadly categorized as red blood cell disor-ders and hemoglobinopathies, white blood cell disorders, platelet disorders, bone marrow disorders, infections and abscesses, cysts and tumors, storage diseases and infiltra-tive disorders, and miscellaneous conditions.4 Inadvertent intraoperative splenic injury is a scenario for which every abdominal surgeon should be prepared. Avail-ability of a predetermined algorithm, with emphasis on the patient’s condition, facilitates intraoperative decision making.5 Partial splenectomy may be a suitable alternative to total splenectomy for certain conditions of hypersplen-ism or splenomegaly, particularly in children in whom preservation of splenic immunologic function is especially important.6 Preoperative splenic artery embolization for elective sple-nectomy has benefits and disadvantages. It may be most suitable in cases of enlarged spleen. Conclusive evidence is lacking.7 Vaccination |
Surgery_Schwartz_10006 | Surgery_Schwartz | splenic artery embolization for elective sple-nectomy has benefits and disadvantages. It may be most suitable in cases of enlarged spleen. Conclusive evidence is lacking.7 Vaccination of the splenectomized patient remains the most effective prevention strategy against OPSI. Preopera-tive vaccination before elective splenectomy is most prudent.8 Laparoscopic splenectomy provides equal hematologic outcomes with decreased morbidity compared with the open operation. The laparoscopic approach has emerged as the standard for elective, nontraumatic splenectomy.9 Overwhelming postsplenectomy infection (OPSI) is an uncommon but potentially grave disease. Children and those undergoing splenectomy for hematologic malig-nancy are at elevated risk.10 Antibiotic prophylactic strategies against OPSI vary widely. Data regarding their use are lacking.Brunicardi_Ch34_p1517-p1548.indd 151823/02/19 2:36 PM 1519THE SPLEENCHAPTER 34The organ continues its differentiation and migration to the left upper | Surgery_Schwartz. splenic artery embolization for elective sple-nectomy has benefits and disadvantages. It may be most suitable in cases of enlarged spleen. Conclusive evidence is lacking.7 Vaccination of the splenectomized patient remains the most effective prevention strategy against OPSI. Preopera-tive vaccination before elective splenectomy is most prudent.8 Laparoscopic splenectomy provides equal hematologic outcomes with decreased morbidity compared with the open operation. The laparoscopic approach has emerged as the standard for elective, nontraumatic splenectomy.9 Overwhelming postsplenectomy infection (OPSI) is an uncommon but potentially grave disease. Children and those undergoing splenectomy for hematologic malig-nancy are at elevated risk.10 Antibiotic prophylactic strategies against OPSI vary widely. Data regarding their use are lacking.Brunicardi_Ch34_p1517-p1548.indd 151823/02/19 2:36 PM 1519THE SPLEENCHAPTER 34The organ continues its differentiation and migration to the left upper |
Surgery_Schwartz_10007 | Surgery_Schwartz | widely. Data regarding their use are lacking.Brunicardi_Ch34_p1517-p1548.indd 151823/02/19 2:36 PM 1519THE SPLEENCHAPTER 34The organ continues its differentiation and migration to the left upper quadrant, where it comes to rest with its smooth, diaphragmatic surface facing posterosuperiorly.16The most common anomaly of splenic embryology is the accessory spleen. Present in up to 20% of the population, one or more accessory spleens may also occur in up to 30% of patients with hematologic disease. Over 80% of accessory spleens are found in the region of the splenic hilum and vascu-lar pedicle. Other locations for accessory spleens in descending order of frequency are the gastrocolic ligament, the pancreas tail, the greater omentum, the stomach’s greater curve, the splenocolic ligament, the small and large bowel mesentery, the left broad ligament in women, and the left spermatic cord in men (Fig. 34-1).10,16The abdominal surface of the diaphragm separates the spleen from the lower | Surgery_Schwartz. widely. Data regarding their use are lacking.Brunicardi_Ch34_p1517-p1548.indd 151823/02/19 2:36 PM 1519THE SPLEENCHAPTER 34The organ continues its differentiation and migration to the left upper quadrant, where it comes to rest with its smooth, diaphragmatic surface facing posterosuperiorly.16The most common anomaly of splenic embryology is the accessory spleen. Present in up to 20% of the population, one or more accessory spleens may also occur in up to 30% of patients with hematologic disease. Over 80% of accessory spleens are found in the region of the splenic hilum and vascu-lar pedicle. Other locations for accessory spleens in descending order of frequency are the gastrocolic ligament, the pancreas tail, the greater omentum, the stomach’s greater curve, the splenocolic ligament, the small and large bowel mesentery, the left broad ligament in women, and the left spermatic cord in men (Fig. 34-1).10,16The abdominal surface of the diaphragm separates the spleen from the lower |
Surgery_Schwartz_10008 | Surgery_Schwartz | the small and large bowel mesentery, the left broad ligament in women, and the left spermatic cord in men (Fig. 34-1).10,16The abdominal surface of the diaphragm separates the spleen from the lower left lung and pleura and the ninth to eleventh ribs. The visceral surface faces the abdominal cavity and contains gastric, colic, renal, and pancreatic impressions. Spleen size and weight vary with age, with both diminishing in the elderly and in those with underlying pathologic conditions. The average adult spleen is 7 to 11 cm in length and weighs 150 g (range, 70–250 g).The spleen’s superior border separates the diaphragmatic surface from the gastric impression of the visceral surface and often contains one or two notches, which are particularly pro-nounced when the spleen is greatly enlarged.Of particular clinical relevance, the spleen is suspended in position by several ligaments and peritoneal folds to the colon (splenocolic ligament), the stomach (gastrosplenic liga-ment), the | Surgery_Schwartz. the small and large bowel mesentery, the left broad ligament in women, and the left spermatic cord in men (Fig. 34-1).10,16The abdominal surface of the diaphragm separates the spleen from the lower left lung and pleura and the ninth to eleventh ribs. The visceral surface faces the abdominal cavity and contains gastric, colic, renal, and pancreatic impressions. Spleen size and weight vary with age, with both diminishing in the elderly and in those with underlying pathologic conditions. The average adult spleen is 7 to 11 cm in length and weighs 150 g (range, 70–250 g).The spleen’s superior border separates the diaphragmatic surface from the gastric impression of the visceral surface and often contains one or two notches, which are particularly pro-nounced when the spleen is greatly enlarged.Of particular clinical relevance, the spleen is suspended in position by several ligaments and peritoneal folds to the colon (splenocolic ligament), the stomach (gastrosplenic liga-ment), the |
Surgery_Schwartz_10009 | Surgery_Schwartz | particular clinical relevance, the spleen is suspended in position by several ligaments and peritoneal folds to the colon (splenocolic ligament), the stomach (gastrosplenic liga-ment), the diaphragm (phrenosplenic ligament), and the kidney, ABCDEFGFigure 34-1. Sites where accessory spleens are found in order of importance. A. Hilar region, 54%; B. pedicle, 25%; C. tail of pan-creas, 6%; D. splenocolic ligament, 2%; E. greater omentum, 12%; F. mesentery, 0.5%; G. left ovary, 0.5%.Gastrosplenic ligamentLesser sacGreater omentumSplenocolic ligamentSustentaculum lienisPhrenicocolic ligamentFigure 34-2. Suspensory ligaments of the spleen.adrenal gland, and tail of the pancreas (splenorenal ligament) (Fig. 34-2). In a related historical footnote it was widely held less than 200 years ago that a “wandering spleen” led women to experience hypochondria. Dietl in 1863 finally clarified that “it was not a patient’s temperament but rather relaxation, exten-sion or the hypoplasia of splenic | Surgery_Schwartz. particular clinical relevance, the spleen is suspended in position by several ligaments and peritoneal folds to the colon (splenocolic ligament), the stomach (gastrosplenic liga-ment), the diaphragm (phrenosplenic ligament), and the kidney, ABCDEFGFigure 34-1. Sites where accessory spleens are found in order of importance. A. Hilar region, 54%; B. pedicle, 25%; C. tail of pan-creas, 6%; D. splenocolic ligament, 2%; E. greater omentum, 12%; F. mesentery, 0.5%; G. left ovary, 0.5%.Gastrosplenic ligamentLesser sacGreater omentumSplenocolic ligamentSustentaculum lienisPhrenicocolic ligamentFigure 34-2. Suspensory ligaments of the spleen.adrenal gland, and tail of the pancreas (splenorenal ligament) (Fig. 34-2). In a related historical footnote it was widely held less than 200 years ago that a “wandering spleen” led women to experience hypochondria. Dietl in 1863 finally clarified that “it was not a patient’s temperament but rather relaxation, exten-sion or the hypoplasia of splenic |
Surgery_Schwartz_10010 | Surgery_Schwartz | that a “wandering spleen” led women to experience hypochondria. Dietl in 1863 finally clarified that “it was not a patient’s temperament but rather relaxation, exten-sion or the hypoplasia of splenic ligaments that made a spleen wander.”17 The gastrosplenic ligament contains the short gas-tric vessels; the remaining ligaments are avascular, with rare exceptions, such as in patients with portal hypertension. The relationship of the pancreas to the spleen also has important clinical implications. In cadaveric anatomic series, the tail of the pancreas has been demonstrated to lie within 1 cm of the splenic hilum 75% of the time and to actually abut the spleen in 30% of patients.2The spleen derives most of its blood from the splenic artery, the longest and most tortuous of the three main branches of the celiac artery. The splenic artery can be characterized by the pattern of its terminal branches. The distributed type of splenic artery is the most common (70%) and is distinguished by a | Surgery_Schwartz. that a “wandering spleen” led women to experience hypochondria. Dietl in 1863 finally clarified that “it was not a patient’s temperament but rather relaxation, exten-sion or the hypoplasia of splenic ligaments that made a spleen wander.”17 The gastrosplenic ligament contains the short gas-tric vessels; the remaining ligaments are avascular, with rare exceptions, such as in patients with portal hypertension. The relationship of the pancreas to the spleen also has important clinical implications. In cadaveric anatomic series, the tail of the pancreas has been demonstrated to lie within 1 cm of the splenic hilum 75% of the time and to actually abut the spleen in 30% of patients.2The spleen derives most of its blood from the splenic artery, the longest and most tortuous of the three main branches of the celiac artery. The splenic artery can be characterized by the pattern of its terminal branches. The distributed type of splenic artery is the most common (70%) and is distinguished by a |
Surgery_Schwartz_10011 | Surgery_Schwartz | of the celiac artery. The splenic artery can be characterized by the pattern of its terminal branches. The distributed type of splenic artery is the most common (70%) and is distinguished by a short trunk with many long branches entering over three-fourths of the spleen’s medial surface. The less common magis-tral type of splenic artery (30%) has a long main trunk dividing near the hilum into short terminal branches, and these enter over 25% to 30% of the spleen’s medial surface. The spleen also receives some of its blood supply from the short gastric vessels that branch from the left gastroepiploic artery running within the gastrosplenic ligament. The splenic vein joins the superior mesenteric vein to form the portal vein and accommodates the major venous drainage of the spleen.When a normal, freshly excised spleen is sectioned, the cut surface is finely granular and predominantly dark red with whit-ish nodules distributed liberally across its expanse. This gross observation reflects | Surgery_Schwartz. of the celiac artery. The splenic artery can be characterized by the pattern of its terminal branches. The distributed type of splenic artery is the most common (70%) and is distinguished by a short trunk with many long branches entering over three-fourths of the spleen’s medial surface. The less common magis-tral type of splenic artery (30%) has a long main trunk dividing near the hilum into short terminal branches, and these enter over 25% to 30% of the spleen’s medial surface. The spleen also receives some of its blood supply from the short gastric vessels that branch from the left gastroepiploic artery running within the gastrosplenic ligament. The splenic vein joins the superior mesenteric vein to form the portal vein and accommodates the major venous drainage of the spleen.When a normal, freshly excised spleen is sectioned, the cut surface is finely granular and predominantly dark red with whit-ish nodules distributed liberally across its expanse. This gross observation reflects |
Surgery_Schwartz_10012 | Surgery_Schwartz | freshly excised spleen is sectioned, the cut surface is finely granular and predominantly dark red with whit-ish nodules distributed liberally across its expanse. This gross observation reflects the spleen’s microstructure. The splenic parenchyma is composed of two main elements: the red pulp, constituting approximately 75% of total splenic volume, and the white pulp (Fig. 34-3). At the interface between the red and white pulp is the narrow marginal zone.Brunicardi_Ch34_p1517-p1548.indd 151923/02/19 2:36 PM 1520SPECIFIC CONSIDERATIONSPART IIBlood enters the red pulp through cords comprised of fibro-blasts and reticular fibers, which contain many macrophages and lack an endothelial lining. The blood then passes from these “open” cords to venous sinuses, which are surrounded and sepa-rated by the same reticulum, and ultimately drains into tributar-ies of the splenic vein. An understanding of the microanatomy of these sinuses has elucidated the mechanical filtration function of the | Surgery_Schwartz. freshly excised spleen is sectioned, the cut surface is finely granular and predominantly dark red with whit-ish nodules distributed liberally across its expanse. This gross observation reflects the spleen’s microstructure. The splenic parenchyma is composed of two main elements: the red pulp, constituting approximately 75% of total splenic volume, and the white pulp (Fig. 34-3). At the interface between the red and white pulp is the narrow marginal zone.Brunicardi_Ch34_p1517-p1548.indd 151923/02/19 2:36 PM 1520SPECIFIC CONSIDERATIONSPART IIBlood enters the red pulp through cords comprised of fibro-blasts and reticular fibers, which contain many macrophages and lack an endothelial lining. The blood then passes from these “open” cords to venous sinuses, which are surrounded and sepa-rated by the same reticulum, and ultimately drains into tributar-ies of the splenic vein. An understanding of the microanatomy of these sinuses has elucidated the mechanical filtration function of the |
Surgery_Schwartz_10013 | Surgery_Schwartz | by the same reticulum, and ultimately drains into tributar-ies of the splenic vein. An understanding of the microanatomy of these sinuses has elucidated the mechanical filtration function of the spleen. Unlike the cords of the red pulp, the sinuses of the red pulp are lined by endothelial cells. These cells contain unique stress fibers that connect the endothelial cells and that contain actin and myosin–like filaments capable of producing a sliding action. When activated, these filaments can create slits or gaps between the endothelial cells through which blood can then pass from the cords.14 Aging erythrocytes with stiffer mem-branes get stuck trying to pass into the sinus and are phagocy-tized by macrophages within the red pulp.15The red pulp thus serves as a dynamic filtration system, enabling macrophages to remove microorganisms, cellular debris, antigen-antibody complexes, and senescent erythrocytes from the circulation.Around the terminal millimeters of splenic arterioles, a | Surgery_Schwartz. by the same reticulum, and ultimately drains into tributar-ies of the splenic vein. An understanding of the microanatomy of these sinuses has elucidated the mechanical filtration function of the spleen. Unlike the cords of the red pulp, the sinuses of the red pulp are lined by endothelial cells. These cells contain unique stress fibers that connect the endothelial cells and that contain actin and myosin–like filaments capable of producing a sliding action. When activated, these filaments can create slits or gaps between the endothelial cells through which blood can then pass from the cords.14 Aging erythrocytes with stiffer mem-branes get stuck trying to pass into the sinus and are phagocy-tized by macrophages within the red pulp.15The red pulp thus serves as a dynamic filtration system, enabling macrophages to remove microorganisms, cellular debris, antigen-antibody complexes, and senescent erythrocytes from the circulation.Around the terminal millimeters of splenic arterioles, a |
Surgery_Schwartz_10014 | Surgery_Schwartz | enabling macrophages to remove microorganisms, cellular debris, antigen-antibody complexes, and senescent erythrocytes from the circulation.Around the terminal millimeters of splenic arterioles, a periarticular lymphatic sheath replaces the native adventitia of the vessel. The sheath is comprised of T lymphocytes and intermittent aggregations of B lymphocytes or lymphoid fol-licles. When antigenically stimulated, the follicles, serving as centers of lymphocyte proliferation, develop germinal centers, which regress as the stimulus or infection subsides. This white pulp consists of nodules that normally are ≤1 mm in size but can increase to several centimeters when nodules coalesce, as occurs in certain lymphoproliferative disorders. At the junction between the white and red pulp is the marginal zone, where lymphocytes are more loosely aggregated.As well as serving as a transit area, the marginal zone is home to its own unique population of cells. Notably two spe-cific types of | Surgery_Schwartz. enabling macrophages to remove microorganisms, cellular debris, antigen-antibody complexes, and senescent erythrocytes from the circulation.Around the terminal millimeters of splenic arterioles, a periarticular lymphatic sheath replaces the native adventitia of the vessel. The sheath is comprised of T lymphocytes and intermittent aggregations of B lymphocytes or lymphoid fol-licles. When antigenically stimulated, the follicles, serving as centers of lymphocyte proliferation, develop germinal centers, which regress as the stimulus or infection subsides. This white pulp consists of nodules that normally are ≤1 mm in size but can increase to several centimeters when nodules coalesce, as occurs in certain lymphoproliferative disorders. At the junction between the white and red pulp is the marginal zone, where lymphocytes are more loosely aggregated.As well as serving as a transit area, the marginal zone is home to its own unique population of cells. Notably two spe-cific types of |
Surgery_Schwartz_10015 | Surgery_Schwartz | the marginal zone, where lymphocytes are more loosely aggregated.As well as serving as a transit area, the marginal zone is home to its own unique population of cells. Notably two spe-cific types of macrophages reside there, marginal zone macro-phages and marginal zone metallophilic macrophages. The former play an important role in the targeting and clearance of certain bacterial pathogens. The latter have been shown to be the main producers of interferons A and B in response to a viral challenge.14PHYSIOLOGY AND PATHOPHYSIOLOGYThe spleen is contained by a 1to 2-mm thick capsule. In humans, the capsule is rich in collagen and contains some elas-tin fibers. Many mammals have splenic capsules and trabecu-lae with abundant smooth muscle cells, which upon autonomic stimulation contract to expel large volumes of stored blood into the general circulation. The human splenic capsule and trabecu-lae, by contrast, contain few or no smooth muscle cells.Total splenic inflow of blood is | Surgery_Schwartz. the marginal zone, where lymphocytes are more loosely aggregated.As well as serving as a transit area, the marginal zone is home to its own unique population of cells. Notably two spe-cific types of macrophages reside there, marginal zone macro-phages and marginal zone metallophilic macrophages. The former play an important role in the targeting and clearance of certain bacterial pathogens. The latter have been shown to be the main producers of interferons A and B in response to a viral challenge.14PHYSIOLOGY AND PATHOPHYSIOLOGYThe spleen is contained by a 1to 2-mm thick capsule. In humans, the capsule is rich in collagen and contains some elas-tin fibers. Many mammals have splenic capsules and trabecu-lae with abundant smooth muscle cells, which upon autonomic stimulation contract to expel large volumes of stored blood into the general circulation. The human splenic capsule and trabecu-lae, by contrast, contain few or no smooth muscle cells.Total splenic inflow of blood is |
Surgery_Schwartz_10016 | Surgery_Schwartz | to expel large volumes of stored blood into the general circulation. The human splenic capsule and trabecu-lae, by contrast, contain few or no smooth muscle cells.Total splenic inflow of blood is approximately 250 to 300 mL/min. Blood flows through successively tapering arteries to arterioles, traverses the white pulp, crosses the marginal zone, and enters the red pulp. From that entry, the flow rate through the spleen may vary greatly. Animal studies measuring the tran-sit times of isotopically labeled blood through the spleen have revealed three distinct velocities of flow. Humans have long been recognized to have both a fast or closed circulation—with blood passing directly from arterioles into venous sinuses—and a slower or open circulation. Most of the spleen’s filtration func-tion occurs via the slower circulation. During open circulation, blood percolates through the reticular space and splenic cords, thus gaining access through gaps or slits in the endothelial cell lining to | Surgery_Schwartz. to expel large volumes of stored blood into the general circulation. The human splenic capsule and trabecu-lae, by contrast, contain few or no smooth muscle cells.Total splenic inflow of blood is approximately 250 to 300 mL/min. Blood flows through successively tapering arteries to arterioles, traverses the white pulp, crosses the marginal zone, and enters the red pulp. From that entry, the flow rate through the spleen may vary greatly. Animal studies measuring the tran-sit times of isotopically labeled blood through the spleen have revealed three distinct velocities of flow. Humans have long been recognized to have both a fast or closed circulation—with blood passing directly from arterioles into venous sinuses—and a slower or open circulation. Most of the spleen’s filtration func-tion occurs via the slower circulation. During open circulation, blood percolates through the reticular space and splenic cords, thus gaining access through gaps or slits in the endothelial cell lining to |
Surgery_Schwartz_10017 | Surgery_Schwartz | occurs via the slower circulation. During open circulation, blood percolates through the reticular space and splenic cords, thus gaining access through gaps or slits in the endothelial cell lining to the sinuses as previously described. Flowing into and out of the venous sinuses through these gaps, the blood is exposed to extensive contact with splenic macrophages. These are responsible for the innate immune response of the spleen, which occurs largely within the marginal zone. The white pulp, by contrast, is involved only in adaptive immunity. In addition, because the passage of plasma through these spaces does not slow in a similar manner, a temporary and unique adhesive con-tact between blood cells and components of the splenic cord may occur. That there is a selective slowing of blood cell flow versus plasma flow is further evidenced by the fact that within the spleen, the erythrocyte concentration (hematocrit) is twice that of the general circulation. During this contact with | Surgery_Schwartz. occurs via the slower circulation. During open circulation, blood percolates through the reticular space and splenic cords, thus gaining access through gaps or slits in the endothelial cell lining to the sinuses as previously described. Flowing into and out of the venous sinuses through these gaps, the blood is exposed to extensive contact with splenic macrophages. These are responsible for the innate immune response of the spleen, which occurs largely within the marginal zone. The white pulp, by contrast, is involved only in adaptive immunity. In addition, because the passage of plasma through these spaces does not slow in a similar manner, a temporary and unique adhesive con-tact between blood cells and components of the splenic cord may occur. That there is a selective slowing of blood cell flow versus plasma flow is further evidenced by the fact that within the spleen, the erythrocyte concentration (hematocrit) is twice that of the general circulation. During this contact with |
Surgery_Schwartz_10018 | Surgery_Schwartz | cell flow versus plasma flow is further evidenced by the fact that within the spleen, the erythrocyte concentration (hematocrit) is twice that of the general circulation. During this contact with splenic macrophages, it is likely that the removal of both cellular debris and senescent blood cells occurs.18The process by which the spleen removes erythrocyte inclusions, such as Heinz bodies (intracellular altered hemo-globin), without cell lysis while red blood cells travel through the spleen is not well understood. The spleen acts as the major site for clearance from the blood of damaged or aged red blood cells and, in addition, has a part in the removal of abnormal white blood cells and platelets. A minimum of 2 days of the erythrocyte’s 120-day life cycle is spent sequestered in the spleen. Daily, approximately 20 mL of aged red blood cells are removed. Evidence suggests that, as erythrocytes age, previ-ously undetected antigens on their surfaces may attach to auto-antibodies in the | Surgery_Schwartz. cell flow versus plasma flow is further evidenced by the fact that within the spleen, the erythrocyte concentration (hematocrit) is twice that of the general circulation. During this contact with splenic macrophages, it is likely that the removal of both cellular debris and senescent blood cells occurs.18The process by which the spleen removes erythrocyte inclusions, such as Heinz bodies (intracellular altered hemo-globin), without cell lysis while red blood cells travel through the spleen is not well understood. The spleen acts as the major site for clearance from the blood of damaged or aged red blood cells and, in addition, has a part in the removal of abnormal white blood cells and platelets. A minimum of 2 days of the erythrocyte’s 120-day life cycle is spent sequestered in the spleen. Daily, approximately 20 mL of aged red blood cells are removed. Evidence suggests that, as erythrocytes age, previ-ously undetected antigens on their surfaces may attach to auto-antibodies in the |
Surgery_Schwartz_10019 | Surgery_Schwartz | Daily, approximately 20 mL of aged red blood cells are removed. Evidence suggests that, as erythrocytes age, previ-ously undetected antigens on their surfaces may attach to auto-antibodies in the circulation; then macrophages may bind to the antibodies and initiate phagocytosis. It is probable that the erythrocyte is damaged over time by multiple passages through the spleen as well as delayed transit through the congested and relatively hypoxic and acidotic environment of the splenic cords.The spleen can also serve as an extra medullary site for hematopoiesis, if required. Another role played by the spleen is in recycling iron. Erythrocytes in large numbers are destroyed intravascularly throughout the body. The released hemoglobin is then bound to haptoglobin, which is ultimately scav-enged from the circulation in the spleen.191Trabeculae (depicted as both capsular and white lined material)Splenic capsuleRed pulpWhite pulpFigure 34-3. Splenic architecture. (Used with permission from | Surgery_Schwartz. Daily, approximately 20 mL of aged red blood cells are removed. Evidence suggests that, as erythrocytes age, previ-ously undetected antigens on their surfaces may attach to auto-antibodies in the circulation; then macrophages may bind to the antibodies and initiate phagocytosis. It is probable that the erythrocyte is damaged over time by multiple passages through the spleen as well as delayed transit through the congested and relatively hypoxic and acidotic environment of the splenic cords.The spleen can also serve as an extra medullary site for hematopoiesis, if required. Another role played by the spleen is in recycling iron. Erythrocytes in large numbers are destroyed intravascularly throughout the body. The released hemoglobin is then bound to haptoglobin, which is ultimately scav-enged from the circulation in the spleen.191Trabeculae (depicted as both capsular and white lined material)Splenic capsuleRed pulpWhite pulpFigure 34-3. Splenic architecture. (Used with permission from |
Surgery_Schwartz_10020 | Surgery_Schwartz | from the circulation in the spleen.191Trabeculae (depicted as both capsular and white lined material)Splenic capsuleRed pulpWhite pulpFigure 34-3. Splenic architecture. (Used with permission from Ivan George, University of Maryland School of Medicine.)Brunicardi_Ch34_p1517-p1548.indd 152023/02/19 2:36 PM 1521THE SPLEENCHAPTER 34The spleen plays a vital, although not indispensable, role in host defense evidenced by the healthy survival of splenec-tomized patients. Both innate and adaptive immune responses (historically categorized as cell-mediated and humoral immu-nity) occur within the spleen.In addition to the previously noted activities of the mar-ginal zone macrophages, marginal zone B cells serve to detect circulating pathogens and respond quickly to either differenti-ate into immunoglobulin M (IgM)–producing plasma cells or to function as antigen-presenting cells (APCs), which facilitate pathogen removal and destruction.It is APC entry in the white pulp in particular that is | Surgery_Schwartz. from the circulation in the spleen.191Trabeculae (depicted as both capsular and white lined material)Splenic capsuleRed pulpWhite pulpFigure 34-3. Splenic architecture. (Used with permission from Ivan George, University of Maryland School of Medicine.)Brunicardi_Ch34_p1517-p1548.indd 152023/02/19 2:36 PM 1521THE SPLEENCHAPTER 34The spleen plays a vital, although not indispensable, role in host defense evidenced by the healthy survival of splenec-tomized patients. Both innate and adaptive immune responses (historically categorized as cell-mediated and humoral immu-nity) occur within the spleen.In addition to the previously noted activities of the mar-ginal zone macrophages, marginal zone B cells serve to detect circulating pathogens and respond quickly to either differenti-ate into immunoglobulin M (IgM)–producing plasma cells or to function as antigen-presenting cells (APCs), which facilitate pathogen removal and destruction.It is APC entry in the white pulp in particular that is |
Surgery_Schwartz_10021 | Surgery_Schwartz | M (IgM)–producing plasma cells or to function as antigen-presenting cells (APCs), which facilitate pathogen removal and destruction.It is APC entry in the white pulp in particular that is key to the initiation of the adaptive immune response. Antigens are thus presented to immunocompetent centers within the lymphoid follicles. This gives rise to the elaboration of immunoglobulins (predominantly IgM). After an antigen challenge, such an acute IgM response results in the release of opsonic antibodies from the white pulp of the spleen. Antigen clearance is then facilitated by the splenic and hepatic reticuloendothelial systems.The structure and immunophysiology of the white pulp is very similar to that of lymph nodes, with the notable difference being that material enters the lymph node in the lymph whereas it is delivered to the white pulp in the blood.20The spleen also produces opsonins, tuftsin, and proper-din. Circulating monocytes are converted within the red pulp into fixed | Surgery_Schwartz. M (IgM)–producing plasma cells or to function as antigen-presenting cells (APCs), which facilitate pathogen removal and destruction.It is APC entry in the white pulp in particular that is key to the initiation of the adaptive immune response. Antigens are thus presented to immunocompetent centers within the lymphoid follicles. This gives rise to the elaboration of immunoglobulins (predominantly IgM). After an antigen challenge, such an acute IgM response results in the release of opsonic antibodies from the white pulp of the spleen. Antigen clearance is then facilitated by the splenic and hepatic reticuloendothelial systems.The structure and immunophysiology of the white pulp is very similar to that of lymph nodes, with the notable difference being that material enters the lymph node in the lymph whereas it is delivered to the white pulp in the blood.20The spleen also produces opsonins, tuftsin, and proper-din. Circulating monocytes are converted within the red pulp into fixed |
Surgery_Schwartz_10022 | Surgery_Schwartz | in the lymph whereas it is delivered to the white pulp in the blood.20The spleen also produces opsonins, tuftsin, and proper-din. Circulating monocytes are converted within the red pulp into fixed macrophages that account for the spleen’s remarkable phagocytic activity.The spleen also appears to be a major source of the protein properdin, important in the initiation of the alternate pathway of complement activation. The splenic reticuloendothelial system is better able to clear bacteria that are poorly or inadequately opsonized from the circulation than is the hepatic reticuloendo-thelial system. Encapsulated bacteria generally fit such a pro-file, hence the risk posed by pneumococcus and Haemophilus influenzae to an asplenic patient. There appears to be sufficient physiologic capacity within the complement cascade to with-stand the loss of tuftsin and properdin production without an increase in patient vulnerability after splenectomy.21-23In patients with chronic hemolytic disorders, | Surgery_Schwartz. in the lymph whereas it is delivered to the white pulp in the blood.20The spleen also produces opsonins, tuftsin, and proper-din. Circulating monocytes are converted within the red pulp into fixed macrophages that account for the spleen’s remarkable phagocytic activity.The spleen also appears to be a major source of the protein properdin, important in the initiation of the alternate pathway of complement activation. The splenic reticuloendothelial system is better able to clear bacteria that are poorly or inadequately opsonized from the circulation than is the hepatic reticuloendo-thelial system. Encapsulated bacteria generally fit such a pro-file, hence the risk posed by pneumococcus and Haemophilus influenzae to an asplenic patient. There appears to be sufficient physiologic capacity within the complement cascade to with-stand the loss of tuftsin and properdin production without an increase in patient vulnerability after splenectomy.21-23In patients with chronic hemolytic disorders, |
Surgery_Schwartz_10023 | Surgery_Schwartz | the complement cascade to with-stand the loss of tuftsin and properdin production without an increase in patient vulnerability after splenectomy.21-23In patients with chronic hemolytic disorders, splenic tissue may become permanently hypertrophied. The reticular spaces of the red pulp become distended with macrophages engorged with the products of erythrocyte breakdown, and splenomegaly can result. It is important to distinguish between splenomegaly and hypersplenism, two similar but distinct terms that are critical to understand when discussing splenic pathology. Splenomegaly refers simply to abnormal enlargement of the spleen. Splenomeg-aly is described variably within the surgical literature as moderate, massive, and hyper, which reflects a lack of con-sensus. Most would agree, however, that splenomegaly applies to organs weighing ≥500 g and/or averaging ≥15 cm in length.Massive splenomegaly similarly lacks a consensus defini-tion but has been described variably as spleens >1 kg in | Surgery_Schwartz. the complement cascade to with-stand the loss of tuftsin and properdin production without an increase in patient vulnerability after splenectomy.21-23In patients with chronic hemolytic disorders, splenic tissue may become permanently hypertrophied. The reticular spaces of the red pulp become distended with macrophages engorged with the products of erythrocyte breakdown, and splenomegaly can result. It is important to distinguish between splenomegaly and hypersplenism, two similar but distinct terms that are critical to understand when discussing splenic pathology. Splenomegaly refers simply to abnormal enlargement of the spleen. Splenomeg-aly is described variably within the surgical literature as moderate, massive, and hyper, which reflects a lack of con-sensus. Most would agree, however, that splenomegaly applies to organs weighing ≥500 g and/or averaging ≥15 cm in length.Massive splenomegaly similarly lacks a consensus defini-tion but has been described variably as spleens >1 kg in |
Surgery_Schwartz_10024 | Surgery_Schwartz | that splenomegaly applies to organs weighing ≥500 g and/or averaging ≥15 cm in length.Massive splenomegaly similarly lacks a consensus defini-tion but has been described variably as spleens >1 kg in mass or >22 cm in length (Fig. 34-4).9 Spleens palpable below the left costal margin are thought to be at least double normal size, with an estimated weight of ≥750 g.24There is not a single, universally accepted standard, but most would agree that an ex vivo mass of >1 kg or a pole-to-pole length of >15 cm generally qualifies as splenomegaly. Hypersplenism often is found in association with splenomegaly but is not synonymous with it. Hypersplenism is defined as the presence of one or more cytopenias in the context of a normally functioning bone marrow.2ABCFigure 34-4. Splenomegaly. A. Computed tomography (CT) scan. B. Three-dimensional reconstruction of CT scan. C. Postoperative specimen.Brunicardi_Ch34_p1517-p1548.indd 152123/02/19 2:36 PM 1522SPECIFIC CONSIDERATIONSPART IIDisorders | Surgery_Schwartz. that splenomegaly applies to organs weighing ≥500 g and/or averaging ≥15 cm in length.Massive splenomegaly similarly lacks a consensus defini-tion but has been described variably as spleens >1 kg in mass or >22 cm in length (Fig. 34-4).9 Spleens palpable below the left costal margin are thought to be at least double normal size, with an estimated weight of ≥750 g.24There is not a single, universally accepted standard, but most would agree that an ex vivo mass of >1 kg or a pole-to-pole length of >15 cm generally qualifies as splenomegaly. Hypersplenism often is found in association with splenomegaly but is not synonymous with it. Hypersplenism is defined as the presence of one or more cytopenias in the context of a normally functioning bone marrow.2ABCFigure 34-4. Splenomegaly. A. Computed tomography (CT) scan. B. Three-dimensional reconstruction of CT scan. C. Postoperative specimen.Brunicardi_Ch34_p1517-p1548.indd 152123/02/19 2:36 PM 1522SPECIFIC CONSIDERATIONSPART IIDisorders |
Surgery_Schwartz_10025 | Surgery_Schwartz | tomography (CT) scan. B. Three-dimensional reconstruction of CT scan. C. Postoperative specimen.Brunicardi_Ch34_p1517-p1548.indd 152123/02/19 2:36 PM 1522SPECIFIC CONSIDERATIONSPART IIDisorders causing hypersplenism can be categorized as either (a) those in which increased destruction of abnormal blood cells occurs in an intrinsically normal spleen (e.g., hemolytic anemias) or (b) primary disorders of the spleen resulting in increased sequestration and destruction of normal blood cells (e.g., infiltrative disorders).The life cycles of cellular elements vary widely in human blood. A neutrophil in circulation has a normal half-life of approximately 6 hours. The spleen’s role in the normal clearance of neutrophils is not well established. It is clear that hypersplen-ism may result in neutropenia through sequestration of normal white blood cells or the removal of abnormal ones. Platelets, on the other hand, generally survive in the circulation for 10 days. Under normal circumstances, | Surgery_Schwartz. tomography (CT) scan. B. Three-dimensional reconstruction of CT scan. C. Postoperative specimen.Brunicardi_Ch34_p1517-p1548.indd 152123/02/19 2:36 PM 1522SPECIFIC CONSIDERATIONSPART IIDisorders causing hypersplenism can be categorized as either (a) those in which increased destruction of abnormal blood cells occurs in an intrinsically normal spleen (e.g., hemolytic anemias) or (b) primary disorders of the spleen resulting in increased sequestration and destruction of normal blood cells (e.g., infiltrative disorders).The life cycles of cellular elements vary widely in human blood. A neutrophil in circulation has a normal half-life of approximately 6 hours. The spleen’s role in the normal clearance of neutrophils is not well established. It is clear that hypersplen-ism may result in neutropenia through sequestration of normal white blood cells or the removal of abnormal ones. Platelets, on the other hand, generally survive in the circulation for 10 days. Under normal circumstances, |
Surgery_Schwartz_10026 | Surgery_Schwartz | through sequestration of normal white blood cells or the removal of abnormal ones. Platelets, on the other hand, generally survive in the circulation for 10 days. Under normal circumstances, a third of the total platelet pool is sequestered in the spleen. Thrombocytopenia may result from excessive sequestration of platelets as well as accelerated platelet destruction in the spleen. Splenomegaly may result in sequestration of up to 80% of the platelet pool. The spleen may also contribute to the immunologic alteration of platelets, which leads to thrombocytopenia in the absence of splenomegaly (e.g., idiopathic thrombocytopenic purpura [ITP]).25The immunologic functions of the spleen are consistent with those of other lymphoid organs. It is a site of bloodborne antigen presentation and the initiation of Tand B-lymphocyte activities involved in humoral and cellular immune responses. Alteration of splenic immune function often gives rise to anti-body production, which results in blood | Surgery_Schwartz. through sequestration of normal white blood cells or the removal of abnormal ones. Platelets, on the other hand, generally survive in the circulation for 10 days. Under normal circumstances, a third of the total platelet pool is sequestered in the spleen. Thrombocytopenia may result from excessive sequestration of platelets as well as accelerated platelet destruction in the spleen. Splenomegaly may result in sequestration of up to 80% of the platelet pool. The spleen may also contribute to the immunologic alteration of platelets, which leads to thrombocytopenia in the absence of splenomegaly (e.g., idiopathic thrombocytopenic purpura [ITP]).25The immunologic functions of the spleen are consistent with those of other lymphoid organs. It is a site of bloodborne antigen presentation and the initiation of Tand B-lymphocyte activities involved in humoral and cellular immune responses. Alteration of splenic immune function often gives rise to anti-body production, which results in blood |
Surgery_Schwartz_10027 | Surgery_Schwartz | initiation of Tand B-lymphocyte activities involved in humoral and cellular immune responses. Alteration of splenic immune function often gives rise to anti-body production, which results in blood cell destruction.Although the spleen contributes to the process of eryth-rocyte maturation, in adult humans there is little evidence of normal hematopoietic function. The spleen does have a minor role in hematopoiesis in the fourth month in the human fetus, and reactivation can occur in childhood if the bone marrow fails to meet the hematologic needs. Splenic hematopoiesis giving rise to abnormal red blood cells is seen in adults with myelopro-liferative disorders. In addition, in response to some anemias, elements of the red pulp may revert to hematopoiesis.INDICATIONS FOR SPLENECTOMYGenerally speaking, splenectomy is performed for the purposes of cure or palliation of hematological disease including condi-tions of hypersplenism, to relieve the mass effect and symptoms associated with | Surgery_Schwartz. initiation of Tand B-lymphocyte activities involved in humoral and cellular immune responses. Alteration of splenic immune function often gives rise to anti-body production, which results in blood cell destruction.Although the spleen contributes to the process of eryth-rocyte maturation, in adult humans there is little evidence of normal hematopoietic function. The spleen does have a minor role in hematopoiesis in the fourth month in the human fetus, and reactivation can occur in childhood if the bone marrow fails to meet the hematologic needs. Splenic hematopoiesis giving rise to abnormal red blood cells is seen in adults with myelopro-liferative disorders. In addition, in response to some anemias, elements of the red pulp may revert to hematopoiesis.INDICATIONS FOR SPLENECTOMYGenerally speaking, splenectomy is performed for the purposes of cure or palliation of hematological disease including condi-tions of hypersplenism, to relieve the mass effect and symptoms associated with |
Surgery_Schwartz_10028 | Surgery_Schwartz | speaking, splenectomy is performed for the purposes of cure or palliation of hematological disease including condi-tions of hypersplenism, to relieve the mass effect and symptoms associated with splenomegaly, to control infection or hemorrhage and finally to diagnose splenic pathology. For the purposes of this chapter, we will divide these indications into the following categories: (a) benign conditions, including red blood cell disor-ders, hemoglobinopathies, and platelet disorders (Table 34-1a); (b) malignant conditions, including white blood cell disorders, bone marrow disorders (myeloproliferative disorders) and tumors of the spleen (Table 34-1b); and (c) miscellaneous conditions and lesions of the spleen including infections and abscesses, cysts, vascular anomalies, and more (Table 34-1c). We will conclude this chapter with a brief discussion on splenic salvage.Overall, the most common indication for splenectomy is trauma to the spleen, whether external trauma (blunt or | Surgery_Schwartz. speaking, splenectomy is performed for the purposes of cure or palliation of hematological disease including condi-tions of hypersplenism, to relieve the mass effect and symptoms associated with splenomegaly, to control infection or hemorrhage and finally to diagnose splenic pathology. For the purposes of this chapter, we will divide these indications into the following categories: (a) benign conditions, including red blood cell disor-ders, hemoglobinopathies, and platelet disorders (Table 34-1a); (b) malignant conditions, including white blood cell disorders, bone marrow disorders (myeloproliferative disorders) and tumors of the spleen (Table 34-1b); and (c) miscellaneous conditions and lesions of the spleen including infections and abscesses, cysts, vascular anomalies, and more (Table 34-1c). We will conclude this chapter with a brief discussion on splenic salvage.Overall, the most common indication for splenectomy is trauma to the spleen, whether external trauma (blunt or |
Surgery_Schwartz_10029 | Surgery_Schwartz | (Table 34-1c). We will conclude this chapter with a brief discussion on splenic salvage.Overall, the most common indication for splenectomy is trauma to the spleen, whether external trauma (blunt or penetrat-ing) or iatrogenic injury (e.g., at the time of other operations). Inadvertent intraoperative injury to the spleen, necessitating removal, also called “incidental splenectomy” is discussed in a 3Table 34-1aIndications for and expected response to splenectomy in various benign diseases, including red blood cell disorders, hemoglobinopathies, and platelet disordersDISEASE/CONDITIONINDICATIONS FOR SPLENECTOMYRESPONSE TO SPLENECTOMYEssential thrombocythemiaOnly for advanced disease (i.e., transformation to myeloid metaplasia or AML) with severe symptomatic splenomegalyRelief of abdominal pain and early satietyGlucose-6-phosphate dehydrogenase deficiency (G6PD)Excessive transfusion requirements; failure of medical therapy (controversial)May be curativeHereditary spherocytosisHemolytic | Surgery_Schwartz. (Table 34-1c). We will conclude this chapter with a brief discussion on splenic salvage.Overall, the most common indication for splenectomy is trauma to the spleen, whether external trauma (blunt or penetrat-ing) or iatrogenic injury (e.g., at the time of other operations). Inadvertent intraoperative injury to the spleen, necessitating removal, also called “incidental splenectomy” is discussed in a 3Table 34-1aIndications for and expected response to splenectomy in various benign diseases, including red blood cell disorders, hemoglobinopathies, and platelet disordersDISEASE/CONDITIONINDICATIONS FOR SPLENECTOMYRESPONSE TO SPLENECTOMYEssential thrombocythemiaOnly for advanced disease (i.e., transformation to myeloid metaplasia or AML) with severe symptomatic splenomegalyRelief of abdominal pain and early satietyGlucose-6-phosphate dehydrogenase deficiency (G6PD)Excessive transfusion requirements; failure of medical therapy (controversial)May be curativeHereditary spherocytosisHemolytic |
Surgery_Schwartz_10030 | Surgery_Schwartz | pain and early satietyGlucose-6-phosphate dehydrogenase deficiency (G6PD)Excessive transfusion requirements; failure of medical therapy (controversial)May be curativeHereditary spherocytosisHemolytic anemia, recurrent transfusions, intractable leg ulcersImproves or eliminates anemiaIdiopathic thrombocytopenic purpura (ITP)Failure of medical therapy, recurrent disease75%–85% rate of long-term responsePolycythemia veraOnly for advanced disease (i.e., transformation to myeloid metaplasia or AML) with severe symptomatic splenomegalyRelief of abdominal pain and early satietyPyruvate kinase deficiencyOnly in severe cases, recurrent transfusionsDecreased transfusion requirement, palliative onlySickle cell diseaseHistory of acute sequestration crisis, splenic symptoms, or infarction (consider concomitant cholecystectomy)Palliative, variable responseThalassemiaExcessive transfusion requirements, symptomatic splenomegaly, infarction, or hypersplenismDiminished transfusion requirements, relief | Surgery_Schwartz. pain and early satietyGlucose-6-phosphate dehydrogenase deficiency (G6PD)Excessive transfusion requirements; failure of medical therapy (controversial)May be curativeHereditary spherocytosisHemolytic anemia, recurrent transfusions, intractable leg ulcersImproves or eliminates anemiaIdiopathic thrombocytopenic purpura (ITP)Failure of medical therapy, recurrent disease75%–85% rate of long-term responsePolycythemia veraOnly for advanced disease (i.e., transformation to myeloid metaplasia or AML) with severe symptomatic splenomegalyRelief of abdominal pain and early satietyPyruvate kinase deficiencyOnly in severe cases, recurrent transfusionsDecreased transfusion requirement, palliative onlySickle cell diseaseHistory of acute sequestration crisis, splenic symptoms, or infarction (consider concomitant cholecystectomy)Palliative, variable responseThalassemiaExcessive transfusion requirements, symptomatic splenomegaly, infarction, or hypersplenismDiminished transfusion requirements, relief |
Surgery_Schwartz_10031 | Surgery_Schwartz | cholecystectomy)Palliative, variable responseThalassemiaExcessive transfusion requirements, symptomatic splenomegaly, infarction, or hypersplenismDiminished transfusion requirements, relief of symptomsThrombotic thrombocytopenic purpura (TTP)Excessive plasma exchange requirementTypically curativeWarm-antibody autoimmune hemolytic anemiaFailure of medical (steroid) therapy60%–80% response rate, recurrences commonBrunicardi_Ch34_p1517-p1548.indd 152223/02/19 2:36 PM 1523THE SPLEENCHAPTER 34Table 34-1bIndications for and expected response to splenectomy in various malignant diseases, including white blood cell disorders, myeloproliferative disorders, and nonhematologic tumors of the spleenDISEASE/CONDITIONINDICATIONS FOR SPLENECTOMYRESPONSE TO SPLENECTOMYAcute myeloid leukemia (AML)Intolerable symptomatic splenomegalyRelief of abdominal pain and early satietyChronic lymphocytic leukemia (CLL)Cytopenias and anemia75% response rateChronic myelogenous leukemia (CML)Symptomatic | Surgery_Schwartz. cholecystectomy)Palliative, variable responseThalassemiaExcessive transfusion requirements, symptomatic splenomegaly, infarction, or hypersplenismDiminished transfusion requirements, relief of symptomsThrombotic thrombocytopenic purpura (TTP)Excessive plasma exchange requirementTypically curativeWarm-antibody autoimmune hemolytic anemiaFailure of medical (steroid) therapy60%–80% response rate, recurrences commonBrunicardi_Ch34_p1517-p1548.indd 152223/02/19 2:36 PM 1523THE SPLEENCHAPTER 34Table 34-1bIndications for and expected response to splenectomy in various malignant diseases, including white blood cell disorders, myeloproliferative disorders, and nonhematologic tumors of the spleenDISEASE/CONDITIONINDICATIONS FOR SPLENECTOMYRESPONSE TO SPLENECTOMYAcute myeloid leukemia (AML)Intolerable symptomatic splenomegalyRelief of abdominal pain and early satietyChronic lymphocytic leukemia (CLL)Cytopenias and anemia75% response rateChronic myelogenous leukemia (CML)Symptomatic |
Surgery_Schwartz_10032 | Surgery_Schwartz | symptomatic splenomegalyRelief of abdominal pain and early satietyChronic lymphocytic leukemia (CLL)Cytopenias and anemia75% response rateChronic myelogenous leukemia (CML)Symptomatic splenomegalyRelief of abdominal pain and early satietyChronic myelomonocytic leukemia (CMML)Symptomatic splenomegalyRelief of abdominal pain and early satietyMyelofibrosis (agnogenic myeloid metaplasia)Severe symptomatic splenomegaly76% clinical response at 1 y, high risk of hemorrhagic, thrombotic, and infectious complications (26%)Hairy cell leukemiaSevere symptomatic splenomegaly, severe transfusion requirements; failure of medical therapyCurativeHodgkin’s lymphomaSurgical staging in selected casesVariedNon-Hodgkin’s lymphomaCytopenias, symptomatic splenomegalyImproved complete blood count values, relief of symptomsMetastatic tumor of the spleen (most commonly breast, lung, and melanoma)If symptomatic, or as part of cancer treatmentVariedPrimary tumor of the spleenFor diagnosis and treatment of | Surgery_Schwartz. symptomatic splenomegalyRelief of abdominal pain and early satietyChronic lymphocytic leukemia (CLL)Cytopenias and anemia75% response rateChronic myelogenous leukemia (CML)Symptomatic splenomegalyRelief of abdominal pain and early satietyChronic myelomonocytic leukemia (CMML)Symptomatic splenomegalyRelief of abdominal pain and early satietyMyelofibrosis (agnogenic myeloid metaplasia)Severe symptomatic splenomegaly76% clinical response at 1 y, high risk of hemorrhagic, thrombotic, and infectious complications (26%)Hairy cell leukemiaSevere symptomatic splenomegaly, severe transfusion requirements; failure of medical therapyCurativeHodgkin’s lymphomaSurgical staging in selected casesVariedNon-Hodgkin’s lymphomaCytopenias, symptomatic splenomegalyImproved complete blood count values, relief of symptomsMetastatic tumor of the spleen (most commonly breast, lung, and melanoma)If symptomatic, or as part of cancer treatmentVariedPrimary tumor of the spleenFor diagnosis and treatment of |
Surgery_Schwartz_10033 | Surgery_Schwartz | of symptomsMetastatic tumor of the spleen (most commonly breast, lung, and melanoma)If symptomatic, or as part of cancer treatmentVariedPrimary tumor of the spleenFor diagnosis and treatment of cancerVariedTable 34-1cIndications for and expected response to splenectomy in various miscellaneous conditionsDISEASE/CONDITIONINDICATIONS FOR SPLENECTOMYRESPONSE TO SPLENECTOMYAbscess of the spleenMultiloculated, or failure of conservative measures for unilocularCurativeAmyloidosisSymptomatic splenomegalyImproves symptoms; does not correct underlying diseaseFelty’s syndromeNeutropenia80% durable response rateGaucher’s diseaseHypersplenismImproves cytopenias; does not correct underlying diseaseNiemann-Pick diseaseSymptomatic splenomegalyImproves symptoms; does not correct underlying diseasePortal/sinistral hypertensionSplenic vein thrombosis, symptomatic splenomegalyPalliativeSarcoidosisHypersplenism or symptomatic splenomegalyImproves symptoms and cytopenias; does not correct underlying | Surgery_Schwartz. of symptomsMetastatic tumor of the spleen (most commonly breast, lung, and melanoma)If symptomatic, or as part of cancer treatmentVariedPrimary tumor of the spleenFor diagnosis and treatment of cancerVariedTable 34-1cIndications for and expected response to splenectomy in various miscellaneous conditionsDISEASE/CONDITIONINDICATIONS FOR SPLENECTOMYRESPONSE TO SPLENECTOMYAbscess of the spleenMultiloculated, or failure of conservative measures for unilocularCurativeAmyloidosisSymptomatic splenomegalyImproves symptoms; does not correct underlying diseaseFelty’s syndromeNeutropenia80% durable response rateGaucher’s diseaseHypersplenismImproves cytopenias; does not correct underlying diseaseNiemann-Pick diseaseSymptomatic splenomegalyImproves symptoms; does not correct underlying diseasePortal/sinistral hypertensionSplenic vein thrombosis, symptomatic splenomegalyPalliativeSarcoidosisHypersplenism or symptomatic splenomegalyImproves symptoms and cytopenias; does not correct underlying |
Surgery_Schwartz_10034 | Surgery_Schwartz | hypertensionSplenic vein thrombosis, symptomatic splenomegalyPalliativeSarcoidosisHypersplenism or symptomatic splenomegalyImproves symptoms and cytopenias; does not correct underlying diseaseSplenic artery aneurysmBest for distal lesions near splenic hilumCurativeSymptomatic nonparasitic cystsPartial splenectomy for small cysts; unroofing for large cystsCurativeSymptomatic parasitic cystsTherapy of choiceCurative; exercise caution not to spill cyst contentsWandering spleenAbdominal pain or splenomegaly (venous congestion)CurativeTraumatic ruptureGrades 4/5, or failure of conservative management of lower gradesCurativeBrunicardi_Ch34_p1517-p1548.indd 152323/02/19 2:36 PM 1524SPECIFIC CONSIDERATIONSPART IIlater section. Management of splenic injury in the trauma patient is also beyond the scope of this chapter and discussed else-where. The most common indications for elective splenec-tomy are malignancy and hematologic autoimmune disorders, principally, idiopathic thrombocytopenic | Surgery_Schwartz. hypertensionSplenic vein thrombosis, symptomatic splenomegalyPalliativeSarcoidosisHypersplenism or symptomatic splenomegalyImproves symptoms and cytopenias; does not correct underlying diseaseSplenic artery aneurysmBest for distal lesions near splenic hilumCurativeSymptomatic nonparasitic cystsPartial splenectomy for small cysts; unroofing for large cystsCurativeSymptomatic parasitic cystsTherapy of choiceCurative; exercise caution not to spill cyst contentsWandering spleenAbdominal pain or splenomegaly (venous congestion)CurativeTraumatic ruptureGrades 4/5, or failure of conservative management of lower gradesCurativeBrunicardi_Ch34_p1517-p1548.indd 152323/02/19 2:36 PM 1524SPECIFIC CONSIDERATIONSPART IIlater section. Management of splenic injury in the trauma patient is also beyond the scope of this chapter and discussed else-where. The most common indications for elective splenec-tomy are malignancy and hematologic autoimmune disorders, principally, idiopathic thrombocytopenic |
Surgery_Schwartz_10035 | Surgery_Schwartz | the scope of this chapter and discussed else-where. The most common indications for elective splenec-tomy are malignancy and hematologic autoimmune disorders, principally, idiopathic thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA).Benign DisordersRed Blood Cell Disorders Congenital Hereditary Spherocytosis. Hereditary spherocytosis (HS) is the most common type of hemolytic anemia for which splenectomy is indicated and the third most common type of congenital hemo-lytic anemia overall.26 HS results from an inherited dysfunction or deficiency in one of the erythrocyte membrane proteins (alpha or beta spectrin, ankyrin, band 3 protein, or protein 4.2). The result-ing destabilization of the membrane lipid bilayer allows a patho-logic release of membrane lipids. The red blood cell assumes a more spherical, less deformable shape, and the spherocytic eryth-rocytes are sequestered and destroyed in the spleen and hemolytic anemia ensues. HS is inherited primarily (70–80% | Surgery_Schwartz. the scope of this chapter and discussed else-where. The most common indications for elective splenec-tomy are malignancy and hematologic autoimmune disorders, principally, idiopathic thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA).Benign DisordersRed Blood Cell Disorders Congenital Hereditary Spherocytosis. Hereditary spherocytosis (HS) is the most common type of hemolytic anemia for which splenectomy is indicated and the third most common type of congenital hemo-lytic anemia overall.26 HS results from an inherited dysfunction or deficiency in one of the erythrocyte membrane proteins (alpha or beta spectrin, ankyrin, band 3 protein, or protein 4.2). The result-ing destabilization of the membrane lipid bilayer allows a patho-logic release of membrane lipids. The red blood cell assumes a more spherical, less deformable shape, and the spherocytic eryth-rocytes are sequestered and destroyed in the spleen and hemolytic anemia ensues. HS is inherited primarily (70–80% |
Surgery_Schwartz_10036 | Surgery_Schwartz | cell assumes a more spherical, less deformable shape, and the spherocytic eryth-rocytes are sequestered and destroyed in the spleen and hemolytic anemia ensues. HS is inherited primarily (70–80% of the time) in an autosomal dominant fashion; the estimated prevalence in Western populations is roughly 1 in 2000.26Patients with typical HS forms may have mild jaundice. Splenomegaly usually is palpable on physical examination. Laboratory examination reveals varying degrees of anemia: patients with mild forms of the disease may not have anemia; patients with moderate to severe forms may have hemoglobin levels as low as 4 to 6 g/dL. The mean corpuscular volume is typically low to normal or slightly decreased. For screening, a combined elevated mean corpuscular hemoglobin concentra-tion and an elevated erythrocyte distribution width are an excel-lent predictor. Other laboratory indicators of HS include those providing evidence of rapid red blood cell destruction, includ-ing elevated | Surgery_Schwartz. cell assumes a more spherical, less deformable shape, and the spherocytic eryth-rocytes are sequestered and destroyed in the spleen and hemolytic anemia ensues. HS is inherited primarily (70–80% of the time) in an autosomal dominant fashion; the estimated prevalence in Western populations is roughly 1 in 2000.26Patients with typical HS forms may have mild jaundice. Splenomegaly usually is palpable on physical examination. Laboratory examination reveals varying degrees of anemia: patients with mild forms of the disease may not have anemia; patients with moderate to severe forms may have hemoglobin levels as low as 4 to 6 g/dL. The mean corpuscular volume is typically low to normal or slightly decreased. For screening, a combined elevated mean corpuscular hemoglobin concentra-tion and an elevated erythrocyte distribution width are an excel-lent predictor. Other laboratory indicators of HS include those providing evidence of rapid red blood cell destruction, includ-ing elevated |
Surgery_Schwartz_10037 | Surgery_Schwartz | and an elevated erythrocyte distribution width are an excel-lent predictor. Other laboratory indicators of HS include those providing evidence of rapid red blood cell destruction, includ-ing elevated reticulocyte count, elevated lactate dehydrogenase level, and increased level of unconjugated bilirubin. Sphero-cytes are readily apparent on peripheral blood film.Risks and benefits should be assessed carefully before splenectomy and cholecystectomy are performed for HS.27 The main indications are moderate to severe symptomatic hemo-lytic anemia, growth retardation, skeletal changes, leg ulcers, and extramedullary hemopoietic tumors in young patients.26,28 If gallstones coexist with spherocytosis, the gallbladder should be removed, but prophylactic cholecystectomy without gall-stones is controversial. Near total splenectomy is advocated in children. Dramatic clinical improvement—despite persistent hemolysis—usually occurs after splenectomy in patients with moderate to severe disease. | Surgery_Schwartz. and an elevated erythrocyte distribution width are an excel-lent predictor. Other laboratory indicators of HS include those providing evidence of rapid red blood cell destruction, includ-ing elevated reticulocyte count, elevated lactate dehydrogenase level, and increased level of unconjugated bilirubin. Sphero-cytes are readily apparent on peripheral blood film.Risks and benefits should be assessed carefully before splenectomy and cholecystectomy are performed for HS.27 The main indications are moderate to severe symptomatic hemo-lytic anemia, growth retardation, skeletal changes, leg ulcers, and extramedullary hemopoietic tumors in young patients.26,28 If gallstones coexist with spherocytosis, the gallbladder should be removed, but prophylactic cholecystectomy without gall-stones is controversial. Near total splenectomy is advocated in children. Dramatic clinical improvement—despite persistent hemolysis—usually occurs after splenectomy in patients with moderate to severe disease. |
Surgery_Schwartz_10038 | Surgery_Schwartz | Near total splenectomy is advocated in children. Dramatic clinical improvement—despite persistent hemolysis—usually occurs after splenectomy in patients with moderate to severe disease. Because children can be affected with HS, the timing of splenectomy is important and is aimed at reducing the diminutive possibility of overwhelming postsple-nectomy sepsis. Delaying such an operation until the patient is between the ages of 4 and 6—unless the anemia and hemolysis accelerate—is recommended by most experts.29Red Blood Cell Enzyme Deficiencies. Red blood cell enzyme deficiencies associated with hemolytic anemia may be classified into two groups: deficiencies of enzymes involved in glycolytic pathways, such as pyruvate kinase deficiency, and deficien-cies of enzymes needed to maintain a high ratio of reduced to oxidized glutathione in the red blood cell, protecting it from oxidative damage, such as glucose-6-phosphate dehydrogenase (G6PD) deficiency.304Pyruvate Kinase Deficiency Pyruvate | Surgery_Schwartz. Near total splenectomy is advocated in children. Dramatic clinical improvement—despite persistent hemolysis—usually occurs after splenectomy in patients with moderate to severe disease. Because children can be affected with HS, the timing of splenectomy is important and is aimed at reducing the diminutive possibility of overwhelming postsple-nectomy sepsis. Delaying such an operation until the patient is between the ages of 4 and 6—unless the anemia and hemolysis accelerate—is recommended by most experts.29Red Blood Cell Enzyme Deficiencies. Red blood cell enzyme deficiencies associated with hemolytic anemia may be classified into two groups: deficiencies of enzymes involved in glycolytic pathways, such as pyruvate kinase deficiency, and deficien-cies of enzymes needed to maintain a high ratio of reduced to oxidized glutathione in the red blood cell, protecting it from oxidative damage, such as glucose-6-phosphate dehydrogenase (G6PD) deficiency.304Pyruvate Kinase Deficiency Pyruvate |
Surgery_Schwartz_10039 | Surgery_Schwartz | ratio of reduced to oxidized glutathione in the red blood cell, protecting it from oxidative damage, such as glucose-6-phosphate dehydrogenase (G6PD) deficiency.304Pyruvate Kinase Deficiency Pyruvate kinase (PK) deficiency is the most common glycolytic defect causing congenital nons-pherocytic hemolytic anemia.31 Since its first description in the early 1960s, vast amounts of information have been elucidated about the genetic diversity of the disease, red blood cell clear-ance, long-term complications and treatment options includ-ing transfusion and splenectomy. PK deficiency affects people worldwide, with a slight preponderance among those of Northern European or Chinese descent. Its estimated prevalence in the Caucasian population is 51 per million.32,33 Clinical manifesta-tions of the disease vary widely, from transfusion-dependent severe anemia in early childhood to well-compensated mild ane-mia in adolescents or adults. Prenatal hydrops fetalis has also been reported.31 Pyruvate | Surgery_Schwartz. ratio of reduced to oxidized glutathione in the red blood cell, protecting it from oxidative damage, such as glucose-6-phosphate dehydrogenase (G6PD) deficiency.304Pyruvate Kinase Deficiency Pyruvate kinase (PK) deficiency is the most common glycolytic defect causing congenital nons-pherocytic hemolytic anemia.31 Since its first description in the early 1960s, vast amounts of information have been elucidated about the genetic diversity of the disease, red blood cell clear-ance, long-term complications and treatment options includ-ing transfusion and splenectomy. PK deficiency affects people worldwide, with a slight preponderance among those of Northern European or Chinese descent. Its estimated prevalence in the Caucasian population is 51 per million.32,33 Clinical manifesta-tions of the disease vary widely, from transfusion-dependent severe anemia in early childhood to well-compensated mild ane-mia in adolescents or adults. Prenatal hydrops fetalis has also been reported.31 Pyruvate |
Surgery_Schwartz_10040 | Surgery_Schwartz | disease vary widely, from transfusion-dependent severe anemia in early childhood to well-compensated mild ane-mia in adolescents or adults. Prenatal hydrops fetalis has also been reported.31 Pyruvate kinase enzyme activity is the gold standard for initial diagnostic testing or by detection of spe-cific mutations at the complementary DNA or genomic level. Splenomegaly is common, and in severe cases, splenectomy can alleviate transfusion requirements.31 As with other disorders that cause hemolytic anemia in children, splenectomy should be delayed if possible to at least 4 years of age to reduce the risk of postsplenectomy infection.Glucose-6-Phosphate Dehydrogenase Deficiency The most common red blood cell enzyme deficiency overall is G6PD deficiency. It is far more prevalent than PK deficiency with more than 400 million people affected worldwide, although most experience only moderate health risks and no longevity reduction.34 Clinical manifestations—chronic hemolytic ane-mia, acute | Surgery_Schwartz. disease vary widely, from transfusion-dependent severe anemia in early childhood to well-compensated mild ane-mia in adolescents or adults. Prenatal hydrops fetalis has also been reported.31 Pyruvate kinase enzyme activity is the gold standard for initial diagnostic testing or by detection of spe-cific mutations at the complementary DNA or genomic level. Splenomegaly is common, and in severe cases, splenectomy can alleviate transfusion requirements.31 As with other disorders that cause hemolytic anemia in children, splenectomy should be delayed if possible to at least 4 years of age to reduce the risk of postsplenectomy infection.Glucose-6-Phosphate Dehydrogenase Deficiency The most common red blood cell enzyme deficiency overall is G6PD deficiency. It is far more prevalent than PK deficiency with more than 400 million people affected worldwide, although most experience only moderate health risks and no longevity reduction.34 Clinical manifestations—chronic hemolytic ane-mia, acute |
Surgery_Schwartz_10041 | Surgery_Schwartz | with more than 400 million people affected worldwide, although most experience only moderate health risks and no longevity reduction.34 Clinical manifestations—chronic hemolytic ane-mia, acute intermittent hemolytic episodes, or no hemolysis—depend on the variant of G6PD deficiency. The mainstay of therapy is avoidance of drugs known to precipitate hemolysis in patients with G6PD deficiency. Transfusions are given in cases of symptomatic anemia. Conventional wisdom is that splenectomy is not indicated in this disease, and certainly the overwhelming majority of patients with G6PD deficiency will neither require nor benefit from splenectomy. However, one report described a small case series of six symptomatic G6PD deficiency patients who had severe hemolytic anemia and required transfusion, all of whom were identified to share a common mutation at exon 10. All underwent splenectomy. A complete response occurred in four patients (transfusion requirement eliminated), and a partial | Surgery_Schwartz. with more than 400 million people affected worldwide, although most experience only moderate health risks and no longevity reduction.34 Clinical manifestations—chronic hemolytic ane-mia, acute intermittent hemolytic episodes, or no hemolysis—depend on the variant of G6PD deficiency. The mainstay of therapy is avoidance of drugs known to precipitate hemolysis in patients with G6PD deficiency. Transfusions are given in cases of symptomatic anemia. Conventional wisdom is that splenectomy is not indicated in this disease, and certainly the overwhelming majority of patients with G6PD deficiency will neither require nor benefit from splenectomy. However, one report described a small case series of six symptomatic G6PD deficiency patients who had severe hemolytic anemia and required transfusion, all of whom were identified to share a common mutation at exon 10. All underwent splenectomy. A complete response occurred in four patients (transfusion requirement eliminated), and a partial |
Surgery_Schwartz_10042 | Surgery_Schwartz | all of whom were identified to share a common mutation at exon 10. All underwent splenectomy. A complete response occurred in four patients (transfusion requirement eliminated), and a partial response occurred in one patient (transfusion requirement reduced); no follow-up data were provided for the remaining patient. This study indi-cates that for a carefully select group of patients with severe hemolytic anemia attributable to G6PD deficiency, splenec-tomy may be of benefit, although more data is needed before strong recommendation can be made.34Acquired Warm-Antibody Autoimmune Hemolytic Anemia. Autoim-mune hemolytic anemias (AIHAs) are characterized by the destruction of red blood cells, whose erythrocyte life span is diminished by autoantibodies leveled against antigens. AIHA is classified as either primary or secondary, depending on whether an underlying cause, such as a disease or toxin, is iden-tified. AIHA is also divided into “warm” and “cold” catego-ries, based on the | Surgery_Schwartz. all of whom were identified to share a common mutation at exon 10. All underwent splenectomy. A complete response occurred in four patients (transfusion requirement eliminated), and a partial response occurred in one patient (transfusion requirement reduced); no follow-up data were provided for the remaining patient. This study indi-cates that for a carefully select group of patients with severe hemolytic anemia attributable to G6PD deficiency, splenec-tomy may be of benefit, although more data is needed before strong recommendation can be made.34Acquired Warm-Antibody Autoimmune Hemolytic Anemia. Autoim-mune hemolytic anemias (AIHAs) are characterized by the destruction of red blood cells, whose erythrocyte life span is diminished by autoantibodies leveled against antigens. AIHA is classified as either primary or secondary, depending on whether an underlying cause, such as a disease or toxin, is iden-tified. AIHA is also divided into “warm” and “cold” catego-ries, based on the |
Surgery_Schwartz_10043 | Surgery_Schwartz | classified as either primary or secondary, depending on whether an underlying cause, such as a disease or toxin, is iden-tified. AIHA is also divided into “warm” and “cold” catego-ries, based on the temperature at which the autoantibodies exert their effect.35 In cold-agglutinin disease, severe symptoms are uncommon and splenectomy is almost never indicated; there-fore, this entity is not discussed further in this section. However, warm-antibody AIHA has clinical consequences with which the surgeon should be familiar.Brunicardi_Ch34_p1517-p1548.indd 152423/02/19 2:36 PM 1525THE SPLEENCHAPTER 34Warm-antibody AIHA, although occurring primarily in midlife, can affect individuals at all ages. The disorder is more common among women, and fully half of warm-antibody AIHA cases are idiopathic. Clinical presentation may be acute or gradual. Findings include mild jaundice and symptoms and signs of anemia. One-third to one-half of patients present with splenomegaly. Sometimes in such cases | Surgery_Schwartz. classified as either primary or secondary, depending on whether an underlying cause, such as a disease or toxin, is iden-tified. AIHA is also divided into “warm” and “cold” catego-ries, based on the temperature at which the autoantibodies exert their effect.35 In cold-agglutinin disease, severe symptoms are uncommon and splenectomy is almost never indicated; there-fore, this entity is not discussed further in this section. However, warm-antibody AIHA has clinical consequences with which the surgeon should be familiar.Brunicardi_Ch34_p1517-p1548.indd 152423/02/19 2:36 PM 1525THE SPLEENCHAPTER 34Warm-antibody AIHA, although occurring primarily in midlife, can affect individuals at all ages. The disorder is more common among women, and fully half of warm-antibody AIHA cases are idiopathic. Clinical presentation may be acute or gradual. Findings include mild jaundice and symptoms and signs of anemia. One-third to one-half of patients present with splenomegaly. Sometimes in such cases |
Surgery_Schwartz_10044 | Surgery_Schwartz | Clinical presentation may be acute or gradual. Findings include mild jaundice and symptoms and signs of anemia. One-third to one-half of patients present with splenomegaly. Sometimes in such cases the spleen is palpable on physical examination. The diagnosis relies on demonstrat-ing hemolysis as indicated by anemia, reticulocytosis, and/or products of red blood cell destruction, including bilirubin, in the blood, urine, and stool. A positive result on direct Coombs’ test confirms the AIHA diagnosis by distinguishing autoimmune from other forms of hemolytic anemia.Treatment of AIHA depends on the severity of the disease and whether it is primary or secondary. Severe symptomatic anemia demands prompt attention, often requiring red blood cell transfusion. The mainstay treatment for both primary and secondary forms of symptomatic, unstable AIHA remains longterm corticosteroid administration.36 Therapy should continue until a response is noted by a rise in hematocrit and fall in | Surgery_Schwartz. Clinical presentation may be acute or gradual. Findings include mild jaundice and symptoms and signs of anemia. One-third to one-half of patients present with splenomegaly. Sometimes in such cases the spleen is palpable on physical examination. The diagnosis relies on demonstrat-ing hemolysis as indicated by anemia, reticulocytosis, and/or products of red blood cell destruction, including bilirubin, in the blood, urine, and stool. A positive result on direct Coombs’ test confirms the AIHA diagnosis by distinguishing autoimmune from other forms of hemolytic anemia.Treatment of AIHA depends on the severity of the disease and whether it is primary or secondary. Severe symptomatic anemia demands prompt attention, often requiring red blood cell transfusion. The mainstay treatment for both primary and secondary forms of symptomatic, unstable AIHA remains longterm corticosteroid administration.36 Therapy should continue until a response is noted by a rise in hematocrit and fall in |
Surgery_Schwartz_10045 | Surgery_Schwartz | both primary and secondary forms of symptomatic, unstable AIHA remains longterm corticosteroid administration.36 Therapy should continue until a response is noted by a rise in hematocrit and fall in reticu-locyte count, which generally occurs within 3 weeks.Clinical response to splenectomy for AIHA varies, and the evidence from a number of small case series is conflicting. For example, one 2004 series reported a favorable response to splenectomy in patients with AIHA secondary to chronic lym-phocytic leukemia, whereas more recent series found no benefit from splenectomy in patients with AIHA secondary to systemic lupus erythematosus or inflammatory bowel disease.37,38Favorable responses to splenectomy have been reported in patients with warm-antibody AIHA, with a recent series show-ing complete remission of refractory AIHA following lapa-roscopic splenectomy at 35-month follow-up in patients over 60 years old.36 Transient responses are more common, how-ever, and many patients | Surgery_Schwartz. both primary and secondary forms of symptomatic, unstable AIHA remains longterm corticosteroid administration.36 Therapy should continue until a response is noted by a rise in hematocrit and fall in reticu-locyte count, which generally occurs within 3 weeks.Clinical response to splenectomy for AIHA varies, and the evidence from a number of small case series is conflicting. For example, one 2004 series reported a favorable response to splenectomy in patients with AIHA secondary to chronic lym-phocytic leukemia, whereas more recent series found no benefit from splenectomy in patients with AIHA secondary to systemic lupus erythematosus or inflammatory bowel disease.37,38Favorable responses to splenectomy have been reported in patients with warm-antibody AIHA, with a recent series show-ing complete remission of refractory AIHA following lapa-roscopic splenectomy at 35-month follow-up in patients over 60 years old.36 Transient responses are more common, how-ever, and many patients |
Surgery_Schwartz_10046 | Surgery_Schwartz | complete remission of refractory AIHA following lapa-roscopic splenectomy at 35-month follow-up in patients over 60 years old.36 Transient responses are more common, how-ever, and many patients eventually experience hemolysis again despite splenectomy.37,38 The decision regarding splenectomy in the case of AIHA should be individualized based on careful consideration of the clinical history and frank discussion with the patient. It is considered as a third-line therapy after failure of steroids or anti-CD20 antibody administration.39Hemoglobinopathies. Sickle cell disease is an inherited chronic hemolytic anemia that results from the mutant sickle cell hemoglobin (HbS) within the red blood cell and is inher-ited in an autosomal dominant fashion. Persons who inherit an HbS gene from one parent (heterozygous) are carriers; those who inherit an HbS gene from both parents (homozygous) have sickle cell anemia.40In sickle cell disease, the underlying abnormality is the mutation of adenine to | Surgery_Schwartz. complete remission of refractory AIHA following lapa-roscopic splenectomy at 35-month follow-up in patients over 60 years old.36 Transient responses are more common, how-ever, and many patients eventually experience hemolysis again despite splenectomy.37,38 The decision regarding splenectomy in the case of AIHA should be individualized based on careful consideration of the clinical history and frank discussion with the patient. It is considered as a third-line therapy after failure of steroids or anti-CD20 antibody administration.39Hemoglobinopathies. Sickle cell disease is an inherited chronic hemolytic anemia that results from the mutant sickle cell hemoglobin (HbS) within the red blood cell and is inher-ited in an autosomal dominant fashion. Persons who inherit an HbS gene from one parent (heterozygous) are carriers; those who inherit an HbS gene from both parents (homozygous) have sickle cell anemia.40In sickle cell disease, the underlying abnormality is the mutation of adenine to |
Surgery_Schwartz_10047 | Surgery_Schwartz | (heterozygous) are carriers; those who inherit an HbS gene from both parents (homozygous) have sickle cell anemia.40In sickle cell disease, the underlying abnormality is the mutation of adenine to thymine in the sixth codon of the β-globin gene, which results in the substitution of valine for glutamic acid as the sixth amino acid of the β-globin chain. Mutant β chains included in the hemoglobin tetramer create HbS. Deoxy-genated HbS is insoluble and becomes polymerized and sickled. The subsequent lack of deformability of the red blood cell, in addition to other processes, results in microvascular congestion, which may lead to thrombosis, ischemia, and tissue necrosis. The disorder is characterized by painful intermittent episodes.Sequestration occurs in the spleen, with splenomegaly resulting early in the disease course. In most patients, subse-quent infarction of the spleen and autosplenectomy occur at some later time. The most frequent indications for splenectomy in sickle cell | Surgery_Schwartz. (heterozygous) are carriers; those who inherit an HbS gene from both parents (homozygous) have sickle cell anemia.40In sickle cell disease, the underlying abnormality is the mutation of adenine to thymine in the sixth codon of the β-globin gene, which results in the substitution of valine for glutamic acid as the sixth amino acid of the β-globin chain. Mutant β chains included in the hemoglobin tetramer create HbS. Deoxy-genated HbS is insoluble and becomes polymerized and sickled. The subsequent lack of deformability of the red blood cell, in addition to other processes, results in microvascular congestion, which may lead to thrombosis, ischemia, and tissue necrosis. The disorder is characterized by painful intermittent episodes.Sequestration occurs in the spleen, with splenomegaly resulting early in the disease course. In most patients, subse-quent infarction of the spleen and autosplenectomy occur at some later time. The most frequent indications for splenectomy in sickle cell |
Surgery_Schwartz_10048 | Surgery_Schwartz | early in the disease course. In most patients, subse-quent infarction of the spleen and autosplenectomy occur at some later time. The most frequent indications for splenectomy in sickle cell disease are recurrent acute sequestration crises, hypersplenism, and splenic abscess. The occurrence of one major acute sequestration crisis, characterized by rapid painful enlargement of the spleen and circulatory collapse, generally is considered sufficient grounds for splenectomy. Both partial and total splenectomy have been shown to control clinical symp-toms in children but may not change hematologic parameters.41 Preoperative preparation should include special attention to adequate hydration and avoidance of hypothermia.Transfusions are often indicated for anemia, for mod-erately severe episodes of acute chest syndrome (i.e., a new infiltrate on chest radiograph associated with new symptoms, such as fever, cough, sputum production, or hypoxia), and pre-operatively before splenectomy. | Surgery_Schwartz. early in the disease course. In most patients, subse-quent infarction of the spleen and autosplenectomy occur at some later time. The most frequent indications for splenectomy in sickle cell disease are recurrent acute sequestration crises, hypersplenism, and splenic abscess. The occurrence of one major acute sequestration crisis, characterized by rapid painful enlargement of the spleen and circulatory collapse, generally is considered sufficient grounds for splenectomy. Both partial and total splenectomy have been shown to control clinical symp-toms in children but may not change hematologic parameters.41 Preoperative preparation should include special attention to adequate hydration and avoidance of hypothermia.Transfusions are often indicated for anemia, for mod-erately severe episodes of acute chest syndrome (i.e., a new infiltrate on chest radiograph associated with new symptoms, such as fever, cough, sputum production, or hypoxia), and pre-operatively before splenectomy. |
Surgery_Schwartz_10049 | Surgery_Schwartz | of acute chest syndrome (i.e., a new infiltrate on chest radiograph associated with new symptoms, such as fever, cough, sputum production, or hypoxia), and pre-operatively before splenectomy. Patients experiencing stroke or a severe crisis may require hydration and an exchange trans-fusion, which may be performed manually or with automated apheresis equipment. Hydroxyurea is an oral chemotherapeutic agent that upregulates fetal hemoglobin, which interferes with polymerization of HbS and thus reduces the sickling process.42Thalassemia. Thalassemia is the term for a group of inherited disorders of hemoglobin synthesis prevalent among people of Mediterranean extraction and classified according to the hemo-globin chain (α, β, or γ) affected. As a group, the thalassemias are the most common genetic diseases known to arise from a single gene defect.43,44 Most forms of this disorder are inherited in Mendelian recessive fashion from asymptomatic carrier par-ents. In the so-called thalassemia | Surgery_Schwartz. of acute chest syndrome (i.e., a new infiltrate on chest radiograph associated with new symptoms, such as fever, cough, sputum production, or hypoxia), and pre-operatively before splenectomy. Patients experiencing stroke or a severe crisis may require hydration and an exchange trans-fusion, which may be performed manually or with automated apheresis equipment. Hydroxyurea is an oral chemotherapeutic agent that upregulates fetal hemoglobin, which interferes with polymerization of HbS and thus reduces the sickling process.42Thalassemia. Thalassemia is the term for a group of inherited disorders of hemoglobin synthesis prevalent among people of Mediterranean extraction and classified according to the hemo-globin chain (α, β, or γ) affected. As a group, the thalassemias are the most common genetic diseases known to arise from a single gene defect.43,44 Most forms of this disorder are inherited in Mendelian recessive fashion from asymptomatic carrier par-ents. In the so-called thalassemia |
Surgery_Schwartz_10050 | Surgery_Schwartz | diseases known to arise from a single gene defect.43,44 Most forms of this disorder are inherited in Mendelian recessive fashion from asymptomatic carrier par-ents. In the so-called thalassemia belt that extends throughout the shores of the Mediterranean as well as through the Arabian Peninsula, Turkey, Iran, India, and southeastern Asia, the inci-dence of thalassemia is between 2.5% and 15%. However, thal-assemias have been found in people of all ethnic origins.44In all forms of thalassemia, the primary defect is absent or reduced production of hemoglobin chains. From this abnormal-ity, two significant consequences arise: (a) reduced functioning of hemoglobin tetramers, yielding hypochromia and microcy-tosis; and (b) unbalanced biosynthesis of individual α and β subunits, which results in insoluble red blood cells that cannot release oxygen normally and may precipitate with cell aging. Both underproduction of hemoglobin and excess production of unpaired hemoglobin subunits contribute | Surgery_Schwartz. diseases known to arise from a single gene defect.43,44 Most forms of this disorder are inherited in Mendelian recessive fashion from asymptomatic carrier par-ents. In the so-called thalassemia belt that extends throughout the shores of the Mediterranean as well as through the Arabian Peninsula, Turkey, Iran, India, and southeastern Asia, the inci-dence of thalassemia is between 2.5% and 15%. However, thal-assemias have been found in people of all ethnic origins.44In all forms of thalassemia, the primary defect is absent or reduced production of hemoglobin chains. From this abnormal-ity, two significant consequences arise: (a) reduced functioning of hemoglobin tetramers, yielding hypochromia and microcy-tosis; and (b) unbalanced biosynthesis of individual α and β subunits, which results in insoluble red blood cells that cannot release oxygen normally and may precipitate with cell aging. Both underproduction of hemoglobin and excess production of unpaired hemoglobin subunits contribute |
Surgery_Schwartz_10051 | Surgery_Schwartz | insoluble red blood cells that cannot release oxygen normally and may precipitate with cell aging. Both underproduction of hemoglobin and excess production of unpaired hemoglobin subunits contribute to thalassemia-associated morbidity and mortality.A diagnosis of thalassemia major (homozygous form) is made by demonstrating hypochromic microcytic anemia asso-ciated with randomly distorted red blood cells and nucleated erythrocytes (target cells) on peripheral blood smear.7 Elevated reticulocyte count and white blood cell count are among the associated findings. Because α chains are needed to form both fetal hemoglobin and adult hemoglobin, α-thalassemia becomes symptomatic in utero or at birth. By contrast, β-thalassemia becomes symptomatic at 4 to 6 months because β chains are involved only in adult hemoglobin synthesis.The clinical spectrum of the thalassemias is wide. Heterozygous carriers of the disease are usually asymptomatic. Homozygous individuals, on the other hand, typically | Surgery_Schwartz. insoluble red blood cells that cannot release oxygen normally and may precipitate with cell aging. Both underproduction of hemoglobin and excess production of unpaired hemoglobin subunits contribute to thalassemia-associated morbidity and mortality.A diagnosis of thalassemia major (homozygous form) is made by demonstrating hypochromic microcytic anemia asso-ciated with randomly distorted red blood cells and nucleated erythrocytes (target cells) on peripheral blood smear.7 Elevated reticulocyte count and white blood cell count are among the associated findings. Because α chains are needed to form both fetal hemoglobin and adult hemoglobin, α-thalassemia becomes symptomatic in utero or at birth. By contrast, β-thalassemia becomes symptomatic at 4 to 6 months because β chains are involved only in adult hemoglobin synthesis.The clinical spectrum of the thalassemias is wide. Heterozygous carriers of the disease are usually asymptomatic. Homozygous individuals, on the other hand, typically |
Surgery_Schwartz_10052 | Surgery_Schwartz | in adult hemoglobin synthesis.The clinical spectrum of the thalassemias is wide. Heterozygous carriers of the disease are usually asymptomatic. Homozygous individuals, on the other hand, typically present before 2 years of age with pallor, growth retardation, jaundice, and abdominal swelling due to liver and spleen enlargement. Among other characteristics of thalassemia major are intractable leg ulcers, head enlargement, frequent infections, and the need for periodic blood transfusions. Untreated individuals usually die in late infancy or early childhood from severe anemia.7Treatment for thalassemia involves red blood cell transfu-sions to maintain a hemoglobin level of >9 mg/dL, along with Brunicardi_Ch34_p1517-p1548.indd 152523/02/19 2:36 PM 1526SPECIFIC CONSIDERATIONSPART IIintensive parenteral chelation therapy with deferoxamine. Sple-nectomy is indicated for patients with excessive transfusion requirements (>200 mL/kg per year), discomfort due to spleno-megaly, or painful | Surgery_Schwartz. in adult hemoglobin synthesis.The clinical spectrum of the thalassemias is wide. Heterozygous carriers of the disease are usually asymptomatic. Homozygous individuals, on the other hand, typically present before 2 years of age with pallor, growth retardation, jaundice, and abdominal swelling due to liver and spleen enlargement. Among other characteristics of thalassemia major are intractable leg ulcers, head enlargement, frequent infections, and the need for periodic blood transfusions. Untreated individuals usually die in late infancy or early childhood from severe anemia.7Treatment for thalassemia involves red blood cell transfu-sions to maintain a hemoglobin level of >9 mg/dL, along with Brunicardi_Ch34_p1517-p1548.indd 152523/02/19 2:36 PM 1526SPECIFIC CONSIDERATIONSPART IIintensive parenteral chelation therapy with deferoxamine. Sple-nectomy is indicated for patients with excessive transfusion requirements (>200 mL/kg per year), discomfort due to spleno-megaly, or painful |
Surgery_Schwartz_10053 | Surgery_Schwartz | parenteral chelation therapy with deferoxamine. Sple-nectomy is indicated for patients with excessive transfusion requirements (>200 mL/kg per year), discomfort due to spleno-megaly, or painful splenic infarction. Careful assessment of the risk-benefit ratio is essential. Thalassemia patients are at high risk for pulmonary hypertension after splenectomy; however, the precise pathophysiology of this sequela remains unclear.45,46 The increase in infectious complications is likely to be due to a coexisting immune deficiency, in large part brought about by iron overload, which may be associated both with the thalas-semia itself and with transfusions. The disproportionately high rate of overwhelming postsplenectomy infection in thalassemia patients has led some investigators to consider partial splenec-tomy in children; some success in reducing mortality has been reported.47 However, splenectomy should be delayed until after the age of 4 years unless it is absolutely necessary.Platelet | Surgery_Schwartz. parenteral chelation therapy with deferoxamine. Sple-nectomy is indicated for patients with excessive transfusion requirements (>200 mL/kg per year), discomfort due to spleno-megaly, or painful splenic infarction. Careful assessment of the risk-benefit ratio is essential. Thalassemia patients are at high risk for pulmonary hypertension after splenectomy; however, the precise pathophysiology of this sequela remains unclear.45,46 The increase in infectious complications is likely to be due to a coexisting immune deficiency, in large part brought about by iron overload, which may be associated both with the thalas-semia itself and with transfusions. The disproportionately high rate of overwhelming postsplenectomy infection in thalassemia patients has led some investigators to consider partial splenec-tomy in children; some success in reducing mortality has been reported.47 However, splenectomy should be delayed until after the age of 4 years unless it is absolutely necessary.Platelet |
Surgery_Schwartz_10054 | Surgery_Schwartz | splenec-tomy in children; some success in reducing mortality has been reported.47 However, splenectomy should be delayed until after the age of 4 years unless it is absolutely necessary.Platelet Disorders Idiopathic Thrombocytopenic Purpura Idiopathic thrombo-cytopenic purpura (ITP), also called immune thrombocytopenic purpura, is an autoimmune disorder characterized by a low platelet count and mucocutaneous and petechial bleeding. The low platelet count stems from premature removal of platelets opsonized by antiplatelet immunoglobulin G autoantibodies produced in the spleen. This clearance occurs through the inter-action of platelet autoantibodies with Fc receptors expressed on tissue macrophages, predominantly in the spleen and liver. The estimated incidence of ITP is 100 persons per million annually, about one-half of whom are children.48 Adult-onset and child-hood-onset ITP are strikingly different in their clinical course and management.Patients with ITP typically present with | Surgery_Schwartz. splenec-tomy in children; some success in reducing mortality has been reported.47 However, splenectomy should be delayed until after the age of 4 years unless it is absolutely necessary.Platelet Disorders Idiopathic Thrombocytopenic Purpura Idiopathic thrombo-cytopenic purpura (ITP), also called immune thrombocytopenic purpura, is an autoimmune disorder characterized by a low platelet count and mucocutaneous and petechial bleeding. The low platelet count stems from premature removal of platelets opsonized by antiplatelet immunoglobulin G autoantibodies produced in the spleen. This clearance occurs through the inter-action of platelet autoantibodies with Fc receptors expressed on tissue macrophages, predominantly in the spleen and liver. The estimated incidence of ITP is 100 persons per million annually, about one-half of whom are children.48 Adult-onset and child-hood-onset ITP are strikingly different in their clinical course and management.Patients with ITP typically present with |
Surgery_Schwartz_10055 | Surgery_Schwartz | million annually, about one-half of whom are children.48 Adult-onset and child-hood-onset ITP are strikingly different in their clinical course and management.Patients with ITP typically present with petechiae or ecchymoses, although some experience major bleeding from the outset. Bleeding may occur from mucosal surfaces in the form of gingival bleeding, epistaxis, menorrhagia, hematuria, or even melena. The severity of bleeding frequently corresponds to the deficiency in platelets: Patients with counts greater than 50,000/mm3 usually present with incidental findings; those with counts between 30,000 and 50,000/mm3 often have easy bruis-ing; those with platelet counts between 10,000 and 30,000/mm3 may develop spontaneous petechiae or ecchymoses; and those with counts less than 10,000/mm3 are at risk for internal bleeding.48 The incidence of major intracranial hemorrhage is approximately 1%, and it usually occurs early in the disease course. The duration of the bleeding helps to | Surgery_Schwartz. million annually, about one-half of whom are children.48 Adult-onset and child-hood-onset ITP are strikingly different in their clinical course and management.Patients with ITP typically present with petechiae or ecchymoses, although some experience major bleeding from the outset. Bleeding may occur from mucosal surfaces in the form of gingival bleeding, epistaxis, menorrhagia, hematuria, or even melena. The severity of bleeding frequently corresponds to the deficiency in platelets: Patients with counts greater than 50,000/mm3 usually present with incidental findings; those with counts between 30,000 and 50,000/mm3 often have easy bruis-ing; those with platelet counts between 10,000 and 30,000/mm3 may develop spontaneous petechiae or ecchymoses; and those with counts less than 10,000/mm3 are at risk for internal bleeding.48 The incidence of major intracranial hemorrhage is approximately 1%, and it usually occurs early in the disease course. The duration of the bleeding helps to |
Surgery_Schwartz_10056 | Surgery_Schwartz | are at risk for internal bleeding.48 The incidence of major intracranial hemorrhage is approximately 1%, and it usually occurs early in the disease course. The duration of the bleeding helps to distinguish acute from chronic forms of ITP. Children often present at a young age (peak age of approximately 5 years) with sudden onset of petechiae or purpura several days to weeks after an infectious illness. In contrast, adults experience a more chronic form of disease with an insidious onset. Splenomegaly is uncommon with ITP in both adults and children, and its occurrence should prompt a search for a separate cause of thrombocytopenia. Up to 10% of children, however, have a palpable spleen tip.Diagnosis of ITP is based on exclusion of other possibili-ties in the presence of a low platelet count and mucocutane-ous bleeding. Other diseases resulting in secondary forms of ITP, such as systemic lupus erythematosus, antiphospholipid syndrome, lymphoproliferative disorders, human | Surgery_Schwartz. are at risk for internal bleeding.48 The incidence of major intracranial hemorrhage is approximately 1%, and it usually occurs early in the disease course. The duration of the bleeding helps to distinguish acute from chronic forms of ITP. Children often present at a young age (peak age of approximately 5 years) with sudden onset of petechiae or purpura several days to weeks after an infectious illness. In contrast, adults experience a more chronic form of disease with an insidious onset. Splenomegaly is uncommon with ITP in both adults and children, and its occurrence should prompt a search for a separate cause of thrombocytopenia. Up to 10% of children, however, have a palpable spleen tip.Diagnosis of ITP is based on exclusion of other possibili-ties in the presence of a low platelet count and mucocutane-ous bleeding. Other diseases resulting in secondary forms of ITP, such as systemic lupus erythematosus, antiphospholipid syndrome, lymphoproliferative disorders, human |
Surgery_Schwartz_10057 | Surgery_Schwartz | platelet count and mucocutane-ous bleeding. Other diseases resulting in secondary forms of ITP, such as systemic lupus erythematosus, antiphospholipid syndrome, lymphoproliferative disorders, human immunodefi-ciency virus (HIV) infection, and hepatitis C, should be identi-fied and treated when present. In addition, any history of use of a drug known to cause thrombocytopenia, such as certain antimicrobials, anti-inflammatories, antihypertensives, and 5Table 34-2Second-line treatment options for immune thrombocytopeniaSplenectomyAdvantages• Effective, time-honored treatment• Most effective response (overall 88%, partial 22%, complete 66%)Disadvantages• Risk of overwhelming postsplenectomy infection• Increased risk of thrombotic events• Morbidity and mortality associated with an operationRituximabAdvantages• Nonsurgical• Good experience since 1999• Initial immediate response 63%• 31% response at 2 yearsDisadvantages• Severe toxicity in 2%–6%Thrombopoietin Receptor | Surgery_Schwartz. platelet count and mucocutane-ous bleeding. Other diseases resulting in secondary forms of ITP, such as systemic lupus erythematosus, antiphospholipid syndrome, lymphoproliferative disorders, human immunodefi-ciency virus (HIV) infection, and hepatitis C, should be identi-fied and treated when present. In addition, any history of use of a drug known to cause thrombocytopenia, such as certain antimicrobials, anti-inflammatories, antihypertensives, and 5Table 34-2Second-line treatment options for immune thrombocytopeniaSplenectomyAdvantages• Effective, time-honored treatment• Most effective response (overall 88%, partial 22%, complete 66%)Disadvantages• Risk of overwhelming postsplenectomy infection• Increased risk of thrombotic events• Morbidity and mortality associated with an operationRituximabAdvantages• Nonsurgical• Good experience since 1999• Initial immediate response 63%• 31% response at 2 yearsDisadvantages• Severe toxicity in 2%–6%Thrombopoietin Receptor |
Surgery_Schwartz_10058 | Surgery_Schwartz | with an operationRituximabAdvantages• Nonsurgical• Good experience since 1999• Initial immediate response 63%• 31% response at 2 yearsDisadvantages• Severe toxicity in 2%–6%Thrombopoietin Receptor AgonistAdvantages• Nonsurgical• Oral agent self-administered weekly• 80% response rateDisadvantages• Long-term treatment required• Potential toxicity• Long-term data lacking at this timeantidepressants, should be sought. In addition to low platelet count, another laboratory finding characteristic of ITP is the presence of large, immature platelets (megakaryocytes) on peripheral blood smear.The usual first line of therapy is oral prednisone at a dos-age of 1.0 to 1.5 mg/kg per day.49,50 No consensus exists as to the optimal duration of steroid therapy, but most responses occur within the first 3 weeks. Response rates range from 50% to 75%, but relapses are common. IV immunoglobulin, given at 1.0 g/kg per day for 2 to 3 days, is indicated for internal bleeding when platelet counts remain less | Surgery_Schwartz. with an operationRituximabAdvantages• Nonsurgical• Good experience since 1999• Initial immediate response 63%• 31% response at 2 yearsDisadvantages• Severe toxicity in 2%–6%Thrombopoietin Receptor AgonistAdvantages• Nonsurgical• Oral agent self-administered weekly• 80% response rateDisadvantages• Long-term treatment required• Potential toxicity• Long-term data lacking at this timeantidepressants, should be sought. In addition to low platelet count, another laboratory finding characteristic of ITP is the presence of large, immature platelets (megakaryocytes) on peripheral blood smear.The usual first line of therapy is oral prednisone at a dos-age of 1.0 to 1.5 mg/kg per day.49,50 No consensus exists as to the optimal duration of steroid therapy, but most responses occur within the first 3 weeks. Response rates range from 50% to 75%, but relapses are common. IV immunoglobulin, given at 1.0 g/kg per day for 2 to 3 days, is indicated for internal bleeding when platelet counts remain less |
Surgery_Schwartz_10059 | Surgery_Schwartz | weeks. Response rates range from 50% to 75%, but relapses are common. IV immunoglobulin, given at 1.0 g/kg per day for 2 to 3 days, is indicated for internal bleeding when platelet counts remain less than 5000/mm3, when extensive pur-pura exists, or to preoperatively boost platelets. IV immunoglob-ulin is thought to impair clearance of immunoglobulin G–coated platelets by competing for binding to tissue macrophage recep-tors. An immediate response is common, but a sustained remis-sion is not. The medical approach to ITP has been modified with the advent of rituximab and thrombopoietin receptor agonists.51 Therapeutic recommendations are summarized in Table 34-2. Splenectomy is selectively indicated for failure of medical ther-apy, for prolonged use of steroids with undesirable effects, and in selected cases after first relapse.7 Prolonged use of steroids can be defined in various ways, but a persistent need for more than 10 to 20 mg/d for 3 to 6 months to maintain a platelet count of | Surgery_Schwartz. weeks. Response rates range from 50% to 75%, but relapses are common. IV immunoglobulin, given at 1.0 g/kg per day for 2 to 3 days, is indicated for internal bleeding when platelet counts remain less than 5000/mm3, when extensive pur-pura exists, or to preoperatively boost platelets. IV immunoglob-ulin is thought to impair clearance of immunoglobulin G–coated platelets by competing for binding to tissue macrophage recep-tors. An immediate response is common, but a sustained remis-sion is not. The medical approach to ITP has been modified with the advent of rituximab and thrombopoietin receptor agonists.51 Therapeutic recommendations are summarized in Table 34-2. Splenectomy is selectively indicated for failure of medical ther-apy, for prolonged use of steroids with undesirable effects, and in selected cases after first relapse.7 Prolonged use of steroids can be defined in various ways, but a persistent need for more than 10 to 20 mg/d for 3 to 6 months to maintain a platelet count of |
Surgery_Schwartz_10060 | Surgery_Schwartz | in selected cases after first relapse.7 Prolonged use of steroids can be defined in various ways, but a persistent need for more than 10 to 20 mg/d for 3 to 6 months to maintain a platelet count of greater than 30,000/mm3 generally prompts referral for sple-nectomy. Splenectomy is an effective option for refractory ITP and provides a permanent response without subsequent need for steroids in 75% to 85% of patients. Two recent reviews and meta-analyses have assessed the global results of splenectomy for ITP, specifically after the use of laparoscopic techniques. Brunicardi_Ch34_p1517-p1548.indd 152623/02/19 2:36 PM 1527THE SPLEENCHAPTER 34In 2004, Kojouri and colleagues reviewed 135 case series from 1966 to 2004, pooling 4955 patients.51 The long-term plate-let count response was assessed, as was the ability to predict response and the incidence of complications. Complete response was achieved in 66% of cases with a follow-up ranging from 1 to 153 months, and complete and partial | Surgery_Schwartz. in selected cases after first relapse.7 Prolonged use of steroids can be defined in various ways, but a persistent need for more than 10 to 20 mg/d for 3 to 6 months to maintain a platelet count of greater than 30,000/mm3 generally prompts referral for sple-nectomy. Splenectomy is an effective option for refractory ITP and provides a permanent response without subsequent need for steroids in 75% to 85% of patients. Two recent reviews and meta-analyses have assessed the global results of splenectomy for ITP, specifically after the use of laparoscopic techniques. Brunicardi_Ch34_p1517-p1548.indd 152623/02/19 2:36 PM 1527THE SPLEENCHAPTER 34In 2004, Kojouri and colleagues reviewed 135 case series from 1966 to 2004, pooling 4955 patients.51 The long-term plate-let count response was assessed, as was the ability to predict response and the incidence of complications. Complete response was achieved in 66% of cases with a follow-up ranging from 1 to 153 months, and complete and partial |
Surgery_Schwartz_10061 | Surgery_Schwartz | as was the ability to predict response and the incidence of complications. Complete response was achieved in 66% of cases with a follow-up ranging from 1 to 153 months, and complete and partial responses occurred in as many as 88% of patients, regardless of the duration of follow-up. They also analyzed 12 preoperative demographic, clinical, and laboratory parameters and found no predictive capability of platelet response in any of them. Mortality was very low (1%), and morbidity was 10%. Limitations of this review included old case series and a low percentage of laparoscopic splenectomies. In a 2009 systemic review involving 1223 patients, Mikhael and colleagues evaluated the shortand long-term outcomes after laparoscopic splenectomy.52 The conversion rate to open sur-gery was 5.6%, and the immediate nonresponder rate was 8.2%; however, eventually a clinical response was achieved in 72% of the patients on long-term follow-up (5 years). The initial con-cerns regarding the potential for | Surgery_Schwartz. as was the ability to predict response and the incidence of complications. Complete response was achieved in 66% of cases with a follow-up ranging from 1 to 153 months, and complete and partial responses occurred in as many as 88% of patients, regardless of the duration of follow-up. They also analyzed 12 preoperative demographic, clinical, and laboratory parameters and found no predictive capability of platelet response in any of them. Mortality was very low (1%), and morbidity was 10%. Limitations of this review included old case series and a low percentage of laparoscopic splenectomies. In a 2009 systemic review involving 1223 patients, Mikhael and colleagues evaluated the shortand long-term outcomes after laparoscopic splenectomy.52 The conversion rate to open sur-gery was 5.6%, and the immediate nonresponder rate was 8.2%; however, eventually a clinical response was achieved in 72% of the patients on long-term follow-up (5 years). The initial con-cerns regarding the potential for |
Surgery_Schwartz_10062 | Surgery_Schwartz | immediate nonresponder rate was 8.2%; however, eventually a clinical response was achieved in 72% of the patients on long-term follow-up (5 years). The initial con-cerns regarding the potential for missing accessory spleens, lon-ger operative times, and increased cost related to laparoscopic versus open splenectomy have been resolved. Laparoscopy is now the approach of choice for elective splenectomy for ITP, with recent studies showing improved short-term results and comparable long-term results to conventional open splenectomy in this condition.53-56In children with ITP, the course is typically self-limited, with durable and complete remission in greater than 70% of patients regardless of therapy. Because of the good prognosis without treatment, the decision to intervene surgically is contro-versial and is largely to obviate intracranial hemorrhage as dis-cussed earlier. Thus, children with typical ITP—and certainly those without hemorrhage—are managed principally by obser-vation, | Surgery_Schwartz. immediate nonresponder rate was 8.2%; however, eventually a clinical response was achieved in 72% of the patients on long-term follow-up (5 years). The initial con-cerns regarding the potential for missing accessory spleens, lon-ger operative times, and increased cost related to laparoscopic versus open splenectomy have been resolved. Laparoscopy is now the approach of choice for elective splenectomy for ITP, with recent studies showing improved short-term results and comparable long-term results to conventional open splenectomy in this condition.53-56In children with ITP, the course is typically self-limited, with durable and complete remission in greater than 70% of patients regardless of therapy. Because of the good prognosis without treatment, the decision to intervene surgically is contro-versial and is largely to obviate intracranial hemorrhage as dis-cussed earlier. Thus, children with typical ITP—and certainly those without hemorrhage—are managed principally by obser-vation, |
Surgery_Schwartz_10063 | Surgery_Schwartz | contro-versial and is largely to obviate intracranial hemorrhage as dis-cussed earlier. Thus, children with typical ITP—and certainly those without hemorrhage—are managed principally by obser-vation, with short-term therapy in select cases.57 Urgent sple-nectomy, in conjunction with aggressive medical therapy, may play a role in the rare circumstance of severe, life-threatening bleeding in both children and adults.Thrombotic Thrombocytopenic Purpura Thrombotic throm-bocytopenic purpura (TTP) is a serious disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and neurologic complications. Abnormal platelet clumping occurs in arterioles and capillaries, reducing the lumen of these vessels and predisposing the patient to microvascular thrombotic epi-sodes. The reduced lumen size also causes shearing stresses on erythrocytes, which leads to deformed red blood cells subject to hemolysis. Hemolysis may also be due in part to sequestration and destruction of | Surgery_Schwartz. contro-versial and is largely to obviate intracranial hemorrhage as dis-cussed earlier. Thus, children with typical ITP—and certainly those without hemorrhage—are managed principally by obser-vation, with short-term therapy in select cases.57 Urgent sple-nectomy, in conjunction with aggressive medical therapy, may play a role in the rare circumstance of severe, life-threatening bleeding in both children and adults.Thrombotic Thrombocytopenic Purpura Thrombotic throm-bocytopenic purpura (TTP) is a serious disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and neurologic complications. Abnormal platelet clumping occurs in arterioles and capillaries, reducing the lumen of these vessels and predisposing the patient to microvascular thrombotic epi-sodes. The reduced lumen size also causes shearing stresses on erythrocytes, which leads to deformed red blood cells subject to hemolysis. Hemolysis may also be due in part to sequestration and destruction of |
Surgery_Schwartz_10064 | Surgery_Schwartz | reduced lumen size also causes shearing stresses on erythrocytes, which leads to deformed red blood cells subject to hemolysis. Hemolysis may also be due in part to sequestration and destruction of erythrocytes in the spleen. Research has dem-onstrated that the underlying abnormality is likely related to the persistence of unusually large multimers of von Willebrand fac-tor associated with platelet clumping in the patient’s blood.58,59TTP occurs in approximately 3.7 individuals per million, but this rare disorder’s dramatic clinical sequelae and favorable response to early therapy demand an understanding of its clini-cal presentation to ensure an early diagnosis. Clinical features of the disorder include petechiae, fever, neurologic symptoms, renal failure, and, infrequently, cardiac symptoms such as heart failure or arrhythmias. Petechial hemorrhages in the lower extremities are the most common presenting sign. Along with fever, patients may experience flu-like symptoms, malaise, or | Surgery_Schwartz. reduced lumen size also causes shearing stresses on erythrocytes, which leads to deformed red blood cells subject to hemolysis. Hemolysis may also be due in part to sequestration and destruction of erythrocytes in the spleen. Research has dem-onstrated that the underlying abnormality is likely related to the persistence of unusually large multimers of von Willebrand fac-tor associated with platelet clumping in the patient’s blood.58,59TTP occurs in approximately 3.7 individuals per million, but this rare disorder’s dramatic clinical sequelae and favorable response to early therapy demand an understanding of its clini-cal presentation to ensure an early diagnosis. Clinical features of the disorder include petechiae, fever, neurologic symptoms, renal failure, and, infrequently, cardiac symptoms such as heart failure or arrhythmias. Petechial hemorrhages in the lower extremities are the most common presenting sign. Along with fever, patients may experience flu-like symptoms, malaise, or |
Surgery_Schwartz_10065 | Surgery_Schwartz | such as heart failure or arrhythmias. Petechial hemorrhages in the lower extremities are the most common presenting sign. Along with fever, patients may experience flu-like symptoms, malaise, or fatigue. Neurologic changes range from generalized headaches to altered mental status, seizures, and even coma. Generally, however, the mere presence of petechiae and thrombocytopenia is sufficient to lead to the diagnosis of TTP and consideration of treatment.The diagnosis is confirmed by the peripheral blood smear, which shows schistocytes, nucleated red blood cells, and baso-philic stippling. Although other conditions such as tight aortic stenosis or prosthetic valves may lead to the presence of schis-tocytes, these conditions generally are not accompanied by thrombocytopenia. TTP may be distinguished from autoimmune causes of thrombocytopenia, such as Evans’ syndrome (ITP and autoimmune hemolytic anemia) or systemic lupus erythemato-sus, by a negative result on Coombs’ test.Plasma exchange | Surgery_Schwartz. such as heart failure or arrhythmias. Petechial hemorrhages in the lower extremities are the most common presenting sign. Along with fever, patients may experience flu-like symptoms, malaise, or fatigue. Neurologic changes range from generalized headaches to altered mental status, seizures, and even coma. Generally, however, the mere presence of petechiae and thrombocytopenia is sufficient to lead to the diagnosis of TTP and consideration of treatment.The diagnosis is confirmed by the peripheral blood smear, which shows schistocytes, nucleated red blood cells, and baso-philic stippling. Although other conditions such as tight aortic stenosis or prosthetic valves may lead to the presence of schis-tocytes, these conditions generally are not accompanied by thrombocytopenia. TTP may be distinguished from autoimmune causes of thrombocytopenia, such as Evans’ syndrome (ITP and autoimmune hemolytic anemia) or systemic lupus erythemato-sus, by a negative result on Coombs’ test.Plasma exchange |
Surgery_Schwartz_10066 | Surgery_Schwartz | from autoimmune causes of thrombocytopenia, such as Evans’ syndrome (ITP and autoimmune hemolytic anemia) or systemic lupus erythemato-sus, by a negative result on Coombs’ test.Plasma exchange is the first-line therapy for TTP. This treatment consists of the daily removal of a single volume of the patient’s plasma and its replacement with fresh-frozen plasma until the thrombocytopenia, anemia, and associated symptoms are corrected. Therapy is then tapered over 1 to 2 weeks.40 Splenectomy should be considered in patients who experience relapse or who require multiple plasma exchanges to control severe symptoms, and generally is well tolerated without sig-nificant morbidity.59,60Malignant ConditionsWhite Blood Cell Disorders. The role of splenectomy in patients with white blood cell disorders varies. As for the myelogenous diseases mentioned previously, splenectomy for white blood cell disorders can be effective therapy for symp-tomatic splenomegaly and hypersplenism, improving some | Surgery_Schwartz. from autoimmune causes of thrombocytopenia, such as Evans’ syndrome (ITP and autoimmune hemolytic anemia) or systemic lupus erythemato-sus, by a negative result on Coombs’ test.Plasma exchange is the first-line therapy for TTP. This treatment consists of the daily removal of a single volume of the patient’s plasma and its replacement with fresh-frozen plasma until the thrombocytopenia, anemia, and associated symptoms are corrected. Therapy is then tapered over 1 to 2 weeks.40 Splenectomy should be considered in patients who experience relapse or who require multiple plasma exchanges to control severe symptoms, and generally is well tolerated without sig-nificant morbidity.59,60Malignant ConditionsWhite Blood Cell Disorders. The role of splenectomy in patients with white blood cell disorders varies. As for the myelogenous diseases mentioned previously, splenectomy for white blood cell disorders can be effective therapy for symp-tomatic splenomegaly and hypersplenism, improving some |
Surgery_Schwartz_10067 | Surgery_Schwartz | varies. As for the myelogenous diseases mentioned previously, splenectomy for white blood cell disorders can be effective therapy for symp-tomatic splenomegaly and hypersplenism, improving some clinical parameters but generally not altering the course of the underlying disease or long-term prognosis. Historically, sple-nectomy has played a role during surgical staging for Hodg-kin’s disease, although this practice has become largely obsolete with the advent of imaging technologies (Computed tomogra-phy [CT] scan and 18F-fluorodeoxyglucose positron emission tomography [18F-FDG PET]).61-63 Careful consideration of the intended benefits of splenectomy must be weighed against the significant perioperative and postsplenectomy risks in this often complex patient population.64Hairy Cell Leukemia Hairy cell leukemia (HCL) is an uncom-mon blood disorder, representing only 2% of all adult leukemias. HCL is characterized by splenomegaly, pancytopenia, and large numbers of abnormal lymphocytes in | Surgery_Schwartz. varies. As for the myelogenous diseases mentioned previously, splenectomy for white blood cell disorders can be effective therapy for symp-tomatic splenomegaly and hypersplenism, improving some clinical parameters but generally not altering the course of the underlying disease or long-term prognosis. Historically, sple-nectomy has played a role during surgical staging for Hodg-kin’s disease, although this practice has become largely obsolete with the advent of imaging technologies (Computed tomogra-phy [CT] scan and 18F-fluorodeoxyglucose positron emission tomography [18F-FDG PET]).61-63 Careful consideration of the intended benefits of splenectomy must be weighed against the significant perioperative and postsplenectomy risks in this often complex patient population.64Hairy Cell Leukemia Hairy cell leukemia (HCL) is an uncom-mon blood disorder, representing only 2% of all adult leukemias. HCL is characterized by splenomegaly, pancytopenia, and large numbers of abnormal lymphocytes in |
Surgery_Schwartz_10068 | Surgery_Schwartz | cell leukemia (HCL) is an uncom-mon blood disorder, representing only 2% of all adult leukemias. HCL is characterized by splenomegaly, pancytopenia, and large numbers of abnormal lymphocytes in the bone marrow. These lymphocytes contain irregular hair-like cytoplasmic projections identifiable on the peripheral smear. Many HCL patients have few symptoms and require no specific therapy. Splenectomy for HCL was historically performed as a palliative procedure, alleviating the symptoms associated with splenomegaly and normalizing peripheral blood counts in the majority of patients, but not leading to morphological bone marrow remissions. However, with the advent of diverse new drugs (e.g., ritux-imab, pentostatin, cladribine), splenectomy has become rarely performed.65 Splenectomy should be considered after exhaustive systemic therapy or for those with nontraumatic rupture, and it has also been used to treat pregnant women with HCL to delay onset of chemotherapy.66Hodgkin’s | Surgery_Schwartz. cell leukemia (HCL) is an uncom-mon blood disorder, representing only 2% of all adult leukemias. HCL is characterized by splenomegaly, pancytopenia, and large numbers of abnormal lymphocytes in the bone marrow. These lymphocytes contain irregular hair-like cytoplasmic projections identifiable on the peripheral smear. Many HCL patients have few symptoms and require no specific therapy. Splenectomy for HCL was historically performed as a palliative procedure, alleviating the symptoms associated with splenomegaly and normalizing peripheral blood counts in the majority of patients, but not leading to morphological bone marrow remissions. However, with the advent of diverse new drugs (e.g., ritux-imab, pentostatin, cladribine), splenectomy has become rarely performed.65 Splenectomy should be considered after exhaustive systemic therapy or for those with nontraumatic rupture, and it has also been used to treat pregnant women with HCL to delay onset of chemotherapy.66Hodgkin’s |
Surgery_Schwartz_10069 | Surgery_Schwartz | should be considered after exhaustive systemic therapy or for those with nontraumatic rupture, and it has also been used to treat pregnant women with HCL to delay onset of chemotherapy.66Hodgkin’s Lymphoma Hodgkin’s Lymphoma (HL) is a dis-order of the lymphoid system characterized by the presence of Reed-Sternberg cells (which actually form the minority of the Hodgkin’s tumor). More than 90% of patients with HL pres-ent with lymphadenopathy above the diaphragm. Lymph nodes can become particularly bulky in the mediastinum, which may result in shortness of breath, cough, or obstructive pneumonia. Brunicardi_Ch34_p1517-p1548.indd 152723/02/19 2:36 PM 1528SPECIFIC CONSIDERATIONSPART IILymphadenopathy below the diaphragm is rare on presen-tation but can arise with disease progression. The spleen is often an occult site of spread, but massive splenomegaly is not common. In addition, large spleens do not necessarily signify involvement.62Four major histologic types exist: lymphocyte | Surgery_Schwartz. should be considered after exhaustive systemic therapy or for those with nontraumatic rupture, and it has also been used to treat pregnant women with HCL to delay onset of chemotherapy.66Hodgkin’s Lymphoma Hodgkin’s Lymphoma (HL) is a dis-order of the lymphoid system characterized by the presence of Reed-Sternberg cells (which actually form the minority of the Hodgkin’s tumor). More than 90% of patients with HL pres-ent with lymphadenopathy above the diaphragm. Lymph nodes can become particularly bulky in the mediastinum, which may result in shortness of breath, cough, or obstructive pneumonia. Brunicardi_Ch34_p1517-p1548.indd 152723/02/19 2:36 PM 1528SPECIFIC CONSIDERATIONSPART IILymphadenopathy below the diaphragm is rare on presen-tation but can arise with disease progression. The spleen is often an occult site of spread, but massive splenomegaly is not common. In addition, large spleens do not necessarily signify involvement.62Four major histologic types exist: lymphocyte |
Surgery_Schwartz_10070 | Surgery_Schwartz | The spleen is often an occult site of spread, but massive splenomegaly is not common. In addition, large spleens do not necessarily signify involvement.62Four major histologic types exist: lymphocyte predomi-nance type, nodular sclerosis type, mixed cellularity type, and lymphocyte depletion type. The histologic type, along with location of disease and symptomatology, influence survival for patients with HD. Stage I disease is limited to one anatomic region; stage II disease is defined by the presence of two or more contiguous or noncontiguous regions on the same side of the diaphragm; stage III disease involves disease on both sides of the diaphragm, but limited to lymph nodes, spleen, and Waldeyer’s ring (the ring of lymphoid tissue formed by the lin-gual, palatine, and nasopharyngeal tonsils); and stage IV disease includes involvement of the bone marrow, lung, liver, skin, gas-trointestinal tract, or any organ or tissue other than the lymph nodes or Waldeyer’s ring.62Staging | Surgery_Schwartz. The spleen is often an occult site of spread, but massive splenomegaly is not common. In addition, large spleens do not necessarily signify involvement.62Four major histologic types exist: lymphocyte predomi-nance type, nodular sclerosis type, mixed cellularity type, and lymphocyte depletion type. The histologic type, along with location of disease and symptomatology, influence survival for patients with HD. Stage I disease is limited to one anatomic region; stage II disease is defined by the presence of two or more contiguous or noncontiguous regions on the same side of the diaphragm; stage III disease involves disease on both sides of the diaphragm, but limited to lymph nodes, spleen, and Waldeyer’s ring (the ring of lymphoid tissue formed by the lin-gual, palatine, and nasopharyngeal tonsils); and stage IV disease includes involvement of the bone marrow, lung, liver, skin, gas-trointestinal tract, or any organ or tissue other than the lymph nodes or Waldeyer’s ring.62Staging |
Surgery_Schwartz_10071 | Surgery_Schwartz | tonsils); and stage IV disease includes involvement of the bone marrow, lung, liver, skin, gas-trointestinal tract, or any organ or tissue other than the lymph nodes or Waldeyer’s ring.62Staging laparotomy for HL is less commonly performed in the current era of minimally invasive surgery and advanced imaging techniques. More liberal use of diagnostic tools (CT scan and 18F-FDG PET) and chemotherapy for patients with HL has drastically reduced the indications for surgical staging. Current indications for surgical staging include clini-cal suspicion of lymphoma without evidence of peripheral disease or patients requiring restaging for suspicion of failure after chemotherapy.62,67Non-Hodgkin’s Lymphoma Non-Hodgkin’s lymphoma (NHL) encompasses all malignancies derived from the lymphoid sys-tem except classic HL.63 A proliferation of any one of the three predominant lymph cell types—natural killer cells, T cells, or B cells—may be included in the category of NHL. Because of the wide net | Surgery_Schwartz. tonsils); and stage IV disease includes involvement of the bone marrow, lung, liver, skin, gas-trointestinal tract, or any organ or tissue other than the lymph nodes or Waldeyer’s ring.62Staging laparotomy for HL is less commonly performed in the current era of minimally invasive surgery and advanced imaging techniques. More liberal use of diagnostic tools (CT scan and 18F-FDG PET) and chemotherapy for patients with HL has drastically reduced the indications for surgical staging. Current indications for surgical staging include clini-cal suspicion of lymphoma without evidence of peripheral disease or patients requiring restaging for suspicion of failure after chemotherapy.62,67Non-Hodgkin’s Lymphoma Non-Hodgkin’s lymphoma (NHL) encompasses all malignancies derived from the lymphoid sys-tem except classic HL.63 A proliferation of any one of the three predominant lymph cell types—natural killer cells, T cells, or B cells—may be included in the category of NHL. Because of the wide net |
Surgery_Schwartz_10072 | Surgery_Schwartz | except classic HL.63 A proliferation of any one of the three predominant lymph cell types—natural killer cells, T cells, or B cells—may be included in the category of NHL. Because of the wide net cast by NHL, the clinical presentations of the dis-orders under its umbrella vary. The subentities of NHL may be clinically classified into nodal or extranodal, as well as indolent, aggressive, and very aggressive groups. Patients with indolent lymphomas may present with mild or no symptoms and seek medical attention for a swollen lymph node, whereas the aggres-sive and very aggressive lymphomas create easily noticeable symptoms, such as pain, swelling due to obstruction of vessels, fever, and night sweats. Surgical staging is no longer indicated for NHL because the combination of history and physical exam-ination, chest radiograph and abdominal/pelvic CT scan, biopsy of involved lymph nodes (including laparoscopically directed nodal and liver biopsies), and bone marrow biopsy is | Surgery_Schwartz. except classic HL.63 A proliferation of any one of the three predominant lymph cell types—natural killer cells, T cells, or B cells—may be included in the category of NHL. Because of the wide net cast by NHL, the clinical presentations of the dis-orders under its umbrella vary. The subentities of NHL may be clinically classified into nodal or extranodal, as well as indolent, aggressive, and very aggressive groups. Patients with indolent lymphomas may present with mild or no symptoms and seek medical attention for a swollen lymph node, whereas the aggres-sive and very aggressive lymphomas create easily noticeable symptoms, such as pain, swelling due to obstruction of vessels, fever, and night sweats. Surgical staging is no longer indicated for NHL because the combination of history and physical exam-ination, chest radiograph and abdominal/pelvic CT scan, biopsy of involved lymph nodes (including laparoscopically directed nodal and liver biopsies), and bone marrow biopsy is |
Surgery_Schwartz_10073 | Surgery_Schwartz | and physical exam-ination, chest radiograph and abdominal/pelvic CT scan, biopsy of involved lymph nodes (including laparoscopically directed nodal and liver biopsies), and bone marrow biopsy is suffi-cient.63 Splenomegaly exists in some, but not all, forms of NHL. Splenectomy does not alter the natural history of the disease, but it is indicated in cases where a diagnosis cannot be established by obtaining peripheral tissue and clinical suspicion remains or for management of symptoms related to an enlarged spleen as well as for improvement of cytopenias.68-72Chronic Lymphocytic Leukemia Chronic lymphocytic leuke-mia (CCL) is currently considered a subtype of NHL. The main characteristic of CLL is a progressive accumulation of old and nonfunctional lymphocytes.63,73 Symptoms of CLL are nonspe-cific and include weakness, fatigue, fever without illness, night sweats, and frequent bacterial and viral infections. The most fre-quent finding is lymphadenopathy. When the spleen is enlarged, | Surgery_Schwartz. and physical exam-ination, chest radiograph and abdominal/pelvic CT scan, biopsy of involved lymph nodes (including laparoscopically directed nodal and liver biopsies), and bone marrow biopsy is suffi-cient.63 Splenomegaly exists in some, but not all, forms of NHL. Splenectomy does not alter the natural history of the disease, but it is indicated in cases where a diagnosis cannot be established by obtaining peripheral tissue and clinical suspicion remains or for management of symptoms related to an enlarged spleen as well as for improvement of cytopenias.68-72Chronic Lymphocytic Leukemia Chronic lymphocytic leuke-mia (CCL) is currently considered a subtype of NHL. The main characteristic of CLL is a progressive accumulation of old and nonfunctional lymphocytes.63,73 Symptoms of CLL are nonspe-cific and include weakness, fatigue, fever without illness, night sweats, and frequent bacterial and viral infections. The most fre-quent finding is lymphadenopathy. When the spleen is enlarged, |
Surgery_Schwartz_10074 | Surgery_Schwartz | and include weakness, fatigue, fever without illness, night sweats, and frequent bacterial and viral infections. The most fre-quent finding is lymphadenopathy. When the spleen is enlarged, it may be massive or barely palpable below the costal margin. Splenectomy is indicated to improve cytopenias and was shown to be 75% effective in a combined group of patients who had either CLL or nonmalignant HD.29 Splenectomy may facilitate chemotherapy in patients whose cell counts were prohibitively low before spleen removal. Palliative splenectomy also is indi-cated for symptomatic splenomegaly.Bone Marrow Disorders (Myeloproliferative Disorders). The myeloproliferative disorders are characterized by an abnor-mal growth of cell lines in the bone marrow. They include chronic myeloid leukemia, acute myeloid leukemia, chronic myelomono-cytic leukemia, essential thrombocythemia, polycythemia vera, and myelofibrosis, also known as agnogenic myeloid metaplasia (see “Myelofibrosis [Agnogenic Myeloid | Surgery_Schwartz. and include weakness, fatigue, fever without illness, night sweats, and frequent bacterial and viral infections. The most fre-quent finding is lymphadenopathy. When the spleen is enlarged, it may be massive or barely palpable below the costal margin. Splenectomy is indicated to improve cytopenias and was shown to be 75% effective in a combined group of patients who had either CLL or nonmalignant HD.29 Splenectomy may facilitate chemotherapy in patients whose cell counts were prohibitively low before spleen removal. Palliative splenectomy also is indi-cated for symptomatic splenomegaly.Bone Marrow Disorders (Myeloproliferative Disorders). The myeloproliferative disorders are characterized by an abnor-mal growth of cell lines in the bone marrow. They include chronic myeloid leukemia, acute myeloid leukemia, chronic myelomono-cytic leukemia, essential thrombocythemia, polycythemia vera, and myelofibrosis, also known as agnogenic myeloid metaplasia (see “Myelofibrosis [Agnogenic Myeloid |
Surgery_Schwartz_10075 | Surgery_Schwartz | myeloid leukemia, chronic myelomono-cytic leukemia, essential thrombocythemia, polycythemia vera, and myelofibrosis, also known as agnogenic myeloid metaplasia (see “Myelofibrosis [Agnogenic Myeloid Metaplasia]” later in this chapter). The common underlying problem leading to sple-nectomy in these disorders is symptomatic splenomegaly. Symp-toms due to splenomegaly are due to mass effect and consist of early satiety, poor gastric emptying, heaviness or pain in the left upper quadrant, and even diarrhea. Hypersplenism, when it occurs in these conditions, usually is associated with splenomeg-aly. Splenectomy performed in the setting of the myeloprolifera-tive disorders is generally for treatment of the pain, early satiety, and other symptoms of splenomegaly. Radiation has been used since 1903 to treat symptomatic splenomegaly in myeloprolif-erative disorders, but today it is principally used in situations in which splenectomy is not an option.Chronic Myelogenous Leukemia Chronic | Surgery_Schwartz. myeloid leukemia, chronic myelomono-cytic leukemia, essential thrombocythemia, polycythemia vera, and myelofibrosis, also known as agnogenic myeloid metaplasia (see “Myelofibrosis [Agnogenic Myeloid Metaplasia]” later in this chapter). The common underlying problem leading to sple-nectomy in these disorders is symptomatic splenomegaly. Symp-toms due to splenomegaly are due to mass effect and consist of early satiety, poor gastric emptying, heaviness or pain in the left upper quadrant, and even diarrhea. Hypersplenism, when it occurs in these conditions, usually is associated with splenomeg-aly. Splenectomy performed in the setting of the myeloprolifera-tive disorders is generally for treatment of the pain, early satiety, and other symptoms of splenomegaly. Radiation has been used since 1903 to treat symptomatic splenomegaly in myeloprolif-erative disorders, but today it is principally used in situations in which splenectomy is not an option.Chronic Myelogenous Leukemia Chronic |
Surgery_Schwartz_10076 | Surgery_Schwartz | 1903 to treat symptomatic splenomegaly in myeloprolif-erative disorders, but today it is principally used in situations in which splenectomy is not an option.Chronic Myelogenous Leukemia Chronic myelogenous leu-kemia (CML) is a disorder of the primitive pluripotent stem cells in the bone marrow that results in a significant increase in erythroid, megakaryotic, and pluripotent progenitors in the peripheral blood smear. The genetic hallmark is a transposition between the bcr gene on chromosome 9 and the abl gene on chromosome 22. CML accounts for 7% to 15% of all leukemias, with an incidence of 1.5 in 100,000 in the United States.44 It is often asymptomatic, but CML can cause fatigue, anorexia, sweating, and left upper quadrant pain and early satiety sec-ondary to splenomegaly. Enlargement of the spleen is found in roughly one-half of patients with CML. Current therapy includes imatinib or allogeneic stem cell transplantation. Splenectomy is indicated to relieve symptoms of massive | Surgery_Schwartz. 1903 to treat symptomatic splenomegaly in myeloprolif-erative disorders, but today it is principally used in situations in which splenectomy is not an option.Chronic Myelogenous Leukemia Chronic myelogenous leu-kemia (CML) is a disorder of the primitive pluripotent stem cells in the bone marrow that results in a significant increase in erythroid, megakaryotic, and pluripotent progenitors in the peripheral blood smear. The genetic hallmark is a transposition between the bcr gene on chromosome 9 and the abl gene on chromosome 22. CML accounts for 7% to 15% of all leukemias, with an incidence of 1.5 in 100,000 in the United States.44 It is often asymptomatic, but CML can cause fatigue, anorexia, sweating, and left upper quadrant pain and early satiety sec-ondary to splenomegaly. Enlargement of the spleen is found in roughly one-half of patients with CML. Current therapy includes imatinib or allogeneic stem cell transplantation. Splenectomy is indicated to relieve symptoms of massive |
Surgery_Schwartz_10077 | Surgery_Schwartz | of the spleen is found in roughly one-half of patients with CML. Current therapy includes imatinib or allogeneic stem cell transplantation. Splenectomy is indicated to relieve symptoms of massive splenomegaly, but it does not prevent blast crisis or alter the disease process.45,70Acute Myeloid Leukemia Like CML, acute myeloid leu-kemia (AML) involves the abnormal growth of stem cells in the bone marrow. Unlike CML, AML has a presentation that is more rapid and dramatic. The proliferation and accumula-tion of hematopoietic stem cells in the bone marrow and blood inhibit the growth and maturation of normal red blood cells, white blood cells, and platelets. Death usually results within weeks to months if AML goes untreated. The incidence of AML is approximately 9200 new cases each year in the United States, and it accounts for 1.2% of all cancer deaths.74 Patients with other myeloproliferative disorders, such as polycythemia vera, primary thrombocytosis, or myeloid metaplasia, are at | Surgery_Schwartz. of the spleen is found in roughly one-half of patients with CML. Current therapy includes imatinib or allogeneic stem cell transplantation. Splenectomy is indicated to relieve symptoms of massive splenomegaly, but it does not prevent blast crisis or alter the disease process.45,70Acute Myeloid Leukemia Like CML, acute myeloid leu-kemia (AML) involves the abnormal growth of stem cells in the bone marrow. Unlike CML, AML has a presentation that is more rapid and dramatic. The proliferation and accumula-tion of hematopoietic stem cells in the bone marrow and blood inhibit the growth and maturation of normal red blood cells, white blood cells, and platelets. Death usually results within weeks to months if AML goes untreated. The incidence of AML is approximately 9200 new cases each year in the United States, and it accounts for 1.2% of all cancer deaths.74 Patients with other myeloproliferative disorders, such as polycythemia vera, primary thrombocytosis, or myeloid metaplasia, are at |
Surgery_Schwartz_10078 | Surgery_Schwartz | the United States, and it accounts for 1.2% of all cancer deaths.74 Patients with other myeloproliferative disorders, such as polycythemia vera, primary thrombocytosis, or myeloid metaplasia, are at increased risk for leukemic transformation to AML. Presenting signs and symptoms of AML include a viral-like illness with fever, malaise, and frequently bone pain due to the expansion of the medullary space. Standard treatment is combined induction therapy with daunorubicin, cytarabine, and stem cell transplan-tation. Splenectomy is indicated in AML only in the uncom-mon circumstance that left upper quadrant pain and early satiety become unbearable. The benefit must be weighed against the heightened risk of postsplenectomy infection in AML patients immunocompromised due to neutropenia and chemotherapy.74Brunicardi_Ch34_p1517-p1548.indd 152823/02/19 2:36 PM 1529THE SPLEENCHAPTER 34Chronic Myelomonocytic Leukemia Like CML and AML, chronic myelomonocytic leukemia (CMML) is characterized | Surgery_Schwartz. the United States, and it accounts for 1.2% of all cancer deaths.74 Patients with other myeloproliferative disorders, such as polycythemia vera, primary thrombocytosis, or myeloid metaplasia, are at increased risk for leukemic transformation to AML. Presenting signs and symptoms of AML include a viral-like illness with fever, malaise, and frequently bone pain due to the expansion of the medullary space. Standard treatment is combined induction therapy with daunorubicin, cytarabine, and stem cell transplan-tation. Splenectomy is indicated in AML only in the uncom-mon circumstance that left upper quadrant pain and early satiety become unbearable. The benefit must be weighed against the heightened risk of postsplenectomy infection in AML patients immunocompromised due to neutropenia and chemotherapy.74Brunicardi_Ch34_p1517-p1548.indd 152823/02/19 2:36 PM 1529THE SPLEENCHAPTER 34Chronic Myelomonocytic Leukemia Like CML and AML, chronic myelomonocytic leukemia (CMML) is characterized |
Surgery_Schwartz_10079 | Surgery_Schwartz | 152823/02/19 2:36 PM 1529THE SPLEENCHAPTER 34Chronic Myelomonocytic Leukemia Like CML and AML, chronic myelomonocytic leukemia (CMML) is characterized by a proliferation of hematopoietic elements in the bone marrow and blood. CMML differs from CML in that it is associated with monocytosis in the peripheral smear (>1 × 103 monocytes/mm3) and in the bone marrow. Splenomegaly occurs in one-half of these patients, and splenectomy can result in symptomatic relief.75Essential Thrombocythemia Essential thrombocythemia (ET) represents abnormal growth of the megakaryocyte cell line, resulting in increased levels of platelets in the bloodstream. The diagnosis is made after the exclusion of other chronic myeloid disorders such as CML, polycythemia vera, and myelofibrosis that may also present with thrombocytosis.76 Clinical manifesta-tions of ET include vasomotor symptoms, thrombohemorrhagic events, recurrent fetal loss, and the transformation to myelofi-brosis with myeloid metaplasia or | Surgery_Schwartz. 152823/02/19 2:36 PM 1529THE SPLEENCHAPTER 34Chronic Myelomonocytic Leukemia Like CML and AML, chronic myelomonocytic leukemia (CMML) is characterized by a proliferation of hematopoietic elements in the bone marrow and blood. CMML differs from CML in that it is associated with monocytosis in the peripheral smear (>1 × 103 monocytes/mm3) and in the bone marrow. Splenomegaly occurs in one-half of these patients, and splenectomy can result in symptomatic relief.75Essential Thrombocythemia Essential thrombocythemia (ET) represents abnormal growth of the megakaryocyte cell line, resulting in increased levels of platelets in the bloodstream. The diagnosis is made after the exclusion of other chronic myeloid disorders such as CML, polycythemia vera, and myelofibrosis that may also present with thrombocytosis.76 Clinical manifesta-tions of ET include vasomotor symptoms, thrombohemorrhagic events, recurrent fetal loss, and the transformation to myelofi-brosis with myeloid metaplasia or |
Surgery_Schwartz_10080 | Surgery_Schwartz | with thrombocytosis.76 Clinical manifesta-tions of ET include vasomotor symptoms, thrombohemorrhagic events, recurrent fetal loss, and the transformation to myelofi-brosis with myeloid metaplasia or AML. Hydroxyurea is used to reduce thrombotic events in ET but does not alter transforma-tion to myelofibrosis or leukemia. Splenomegaly occurs in one-third to one-half of patients with ET. Splenectomy is not felt to be helpful in the early stages of ET and is best reserved for the later stages of disease, when myeloid metaplasia has devel-oped.69 Even in these circumstances, candidates should be cho-sen selectively because significant bleeding has been reported to complicate splenectomy in these patients.Polycythemia Vera Polycythemia vera (PV) is a clonal, chronic, progressive myeloproliferative disorder characterized by an increase in red blood cell mass, frequently accompanied by leukocytosis, thrombocytosis, and splenomegaly. Patients with PV typically enjoy longer survival than those | Surgery_Schwartz. with thrombocytosis.76 Clinical manifesta-tions of ET include vasomotor symptoms, thrombohemorrhagic events, recurrent fetal loss, and the transformation to myelofi-brosis with myeloid metaplasia or AML. Hydroxyurea is used to reduce thrombotic events in ET but does not alter transforma-tion to myelofibrosis or leukemia. Splenomegaly occurs in one-third to one-half of patients with ET. Splenectomy is not felt to be helpful in the early stages of ET and is best reserved for the later stages of disease, when myeloid metaplasia has devel-oped.69 Even in these circumstances, candidates should be cho-sen selectively because significant bleeding has been reported to complicate splenectomy in these patients.Polycythemia Vera Polycythemia vera (PV) is a clonal, chronic, progressive myeloproliferative disorder characterized by an increase in red blood cell mass, frequently accompanied by leukocytosis, thrombocytosis, and splenomegaly. Patients with PV typically enjoy longer survival than those |
Surgery_Schwartz_10081 | Surgery_Schwartz | disorder characterized by an increase in red blood cell mass, frequently accompanied by leukocytosis, thrombocytosis, and splenomegaly. Patients with PV typically enjoy longer survival than those affected by hematologic malignancies but remain at risk for transformation to myelofibrosis or AML. The disease is rare, with an annual incidence of 5 to 17 cases per million population.76,77 Physical findings include ruddy cyanosis, conjunctival plethora, hepato-megaly, splenomegaly, and hypertension. Treatment should be tailored to the risk status of the patient and ranges from phle-botomy and aspirin administration to the use of chemothera-peutic agents. As in ET, splenectomy is not helpful in the early stages of disease and is best reserved for patients with late-stage disease in whom myeloid metaplasia has developed and spleno-megaly-related symptoms are severe.76,77Myelofibrosis (Agnogenic Myeloid Metaplasia) The term myelofibrosis may be used to describe either the generic condi-tion | Surgery_Schwartz. disorder characterized by an increase in red blood cell mass, frequently accompanied by leukocytosis, thrombocytosis, and splenomegaly. Patients with PV typically enjoy longer survival than those affected by hematologic malignancies but remain at risk for transformation to myelofibrosis or AML. The disease is rare, with an annual incidence of 5 to 17 cases per million population.76,77 Physical findings include ruddy cyanosis, conjunctival plethora, hepato-megaly, splenomegaly, and hypertension. Treatment should be tailored to the risk status of the patient and ranges from phle-botomy and aspirin administration to the use of chemothera-peutic agents. As in ET, splenectomy is not helpful in the early stages of disease and is best reserved for patients with late-stage disease in whom myeloid metaplasia has developed and spleno-megaly-related symptoms are severe.76,77Myelofibrosis (Agnogenic Myeloid Metaplasia) The term myelofibrosis may be used to describe either the generic condi-tion |
Surgery_Schwartz_10082 | Surgery_Schwartz | metaplasia has developed and spleno-megaly-related symptoms are severe.76,77Myelofibrosis (Agnogenic Myeloid Metaplasia) The term myelofibrosis may be used to describe either the generic condi-tion of fibrosis of the bone marrow (which may be associated with a number of benign and malignant disorders) or a specific, chronic, malignant hematologic disease associated with sple-nomegaly, the presence of red blood cell and white blood cell progenitors in the bloodstream, marrow fibrosis, and extramed-ullary hematopoiesis, otherwise known as agnogenic myeloid metaplasia (AMM). AMM also can be referred to as myeloscle-rosis, idiopathic myeloid metaplasia, and osteosclerosis. In this chapter, the term myelofibrosis is synonymous with AMM.In AMM, fibrosis of the bone marrow is believed to be a response to a clonal proliferation of hematopoietic stem cells. Marrow failure is common. The true incidence of AMM is unknown due to the scarcity of epidemiologic data, but one study estimated its U.S. | Surgery_Schwartz. metaplasia has developed and spleno-megaly-related symptoms are severe.76,77Myelofibrosis (Agnogenic Myeloid Metaplasia) The term myelofibrosis may be used to describe either the generic condi-tion of fibrosis of the bone marrow (which may be associated with a number of benign and malignant disorders) or a specific, chronic, malignant hematologic disease associated with sple-nomegaly, the presence of red blood cell and white blood cell progenitors in the bloodstream, marrow fibrosis, and extramed-ullary hematopoiesis, otherwise known as agnogenic myeloid metaplasia (AMM). AMM also can be referred to as myeloscle-rosis, idiopathic myeloid metaplasia, and osteosclerosis. In this chapter, the term myelofibrosis is synonymous with AMM.In AMM, fibrosis of the bone marrow is believed to be a response to a clonal proliferation of hematopoietic stem cells. Marrow failure is common. The true incidence of AMM is unknown due to the scarcity of epidemiologic data, but one study estimated its U.S. |
Surgery_Schwartz_10083 | Surgery_Schwartz | to a clonal proliferation of hematopoietic stem cells. Marrow failure is common. The true incidence of AMM is unknown due to the scarcity of epidemiologic data, but one study estimated its U.S. incidence at 1.46 per 100,000 popula-tion.78-80 The diagnosis is made by a careful examination of the peripheral blood smear and bone marrow. Nucleated red blood cells and immature myeloid elements in the blood are present in 96% of cases and strongly suggest the diagnosis. Teardrop poikilocytosis is another frequent finding. Care must be taken, however, to exclude a history of a primary neoplasm (such as lymphoma or adenocarcinoma of the stomach, lung, prostate, or breast) or tuberculosis because patients with these conditions may develop secondary myelofibrosis.Treatment depends on symptoms: Asymptomatic patients are closely followed, whereas symptomatic patients undergo therapeutic intervention targeted to their symptoms. The only curative therapy is allogeneic bone marrow transplantation in | Surgery_Schwartz. to a clonal proliferation of hematopoietic stem cells. Marrow failure is common. The true incidence of AMM is unknown due to the scarcity of epidemiologic data, but one study estimated its U.S. incidence at 1.46 per 100,000 popula-tion.78-80 The diagnosis is made by a careful examination of the peripheral blood smear and bone marrow. Nucleated red blood cells and immature myeloid elements in the blood are present in 96% of cases and strongly suggest the diagnosis. Teardrop poikilocytosis is another frequent finding. Care must be taken, however, to exclude a history of a primary neoplasm (such as lymphoma or adenocarcinoma of the stomach, lung, prostate, or breast) or tuberculosis because patients with these conditions may develop secondary myelofibrosis.Treatment depends on symptoms: Asymptomatic patients are closely followed, whereas symptomatic patients undergo therapeutic intervention targeted to their symptoms. The only curative therapy is allogeneic bone marrow transplantation in |
Surgery_Schwartz_10084 | Surgery_Schwartz | patients are closely followed, whereas symptomatic patients undergo therapeutic intervention targeted to their symptoms. The only curative therapy is allogeneic bone marrow transplantation in younger, high-risk patients. Supportive therapy for clinically symptomatic anemia includes steroids, danazol, erythropoietin, or blood transfusion.78,80 Splenomegaly-related symptoms are best treated with splenectomy. Although some chemotherapeu-tic agents (busulfan, hydroxyurea, interferon-α) and low-dose radiation can reduce splenic size, their discontinuation usually results in rapid splenic regrowth.A thorough preoperative workup must precede sple-nectomy in patients with AMM. The candidate must possess acceptable cardiac, pulmonary, hepatic, and renal reserve for the operation. The coagulation system should be examined; testing should include measurement of coagulation factors V and VIII and fibrin split products, platelet count, and bleeding time. Low platelet counts may require | Surgery_Schwartz. patients are closely followed, whereas symptomatic patients undergo therapeutic intervention targeted to their symptoms. The only curative therapy is allogeneic bone marrow transplantation in younger, high-risk patients. Supportive therapy for clinically symptomatic anemia includes steroids, danazol, erythropoietin, or blood transfusion.78,80 Splenomegaly-related symptoms are best treated with splenectomy. Although some chemotherapeu-tic agents (busulfan, hydroxyurea, interferon-α) and low-dose radiation can reduce splenic size, their discontinuation usually results in rapid splenic regrowth.A thorough preoperative workup must precede sple-nectomy in patients with AMM. The candidate must possess acceptable cardiac, pulmonary, hepatic, and renal reserve for the operation. The coagulation system should be examined; testing should include measurement of coagulation factors V and VIII and fibrin split products, platelet count, and bleeding time. Low platelet counts may require |
Surgery_Schwartz_10085 | Surgery_Schwartz | system should be examined; testing should include measurement of coagulation factors V and VIII and fibrin split products, platelet count, and bleeding time. Low platelet counts may require administration of ste-roids and/or platelet transfusion at the time of surgery. Sple-nectomy provides durable, effective palliation for nearly all patients with AMM, although postoperative complications are more common in patients with AMM than in those with other hematologic indications. The Mayo Clinic recently published its 30-year experience with 314 myelofibrosis patients who underwent splenectomy. Nearly half of the operations (49%) were performed to alleviate the mechanical symptoms of sple-nomegaly; the remainder were undertaken to manage anemia, thrombocytopenia, or portal hypertension. Response to splenec-tomy was 76% overall at 1 year; overall complication rate was 28%, including 21 perioperative deaths.80 Thrombosis, hemor-rhage, and infection complications were common, with | Surgery_Schwartz. system should be examined; testing should include measurement of coagulation factors V and VIII and fibrin split products, platelet count, and bleeding time. Low platelet counts may require administration of ste-roids and/or platelet transfusion at the time of surgery. Sple-nectomy provides durable, effective palliation for nearly all patients with AMM, although postoperative complications are more common in patients with AMM than in those with other hematologic indications. The Mayo Clinic recently published its 30-year experience with 314 myelofibrosis patients who underwent splenectomy. Nearly half of the operations (49%) were performed to alleviate the mechanical symptoms of sple-nomegaly; the remainder were undertaken to manage anemia, thrombocytopenia, or portal hypertension. Response to splenec-tomy was 76% overall at 1 year; overall complication rate was 28%, including 21 perioperative deaths.80 Thrombosis, hemor-rhage, and infection complications were common, with |
Surgery_Schwartz_10086 | Surgery_Schwartz | Response to splenec-tomy was 76% overall at 1 year; overall complication rate was 28%, including 21 perioperative deaths.80 Thrombosis, hemor-rhage, and infection complications were common, with preop-erative thrombocytopenia an independent predictor of mortality risk. These data underscore the severity of this malignancy and emphasize the need for careful patient selection when consider-ing splenectomy in AMM.79Tumors and Metastasis. Primary tumors of the spleen are typ-ically benign or malignant variants of vascular neoplasms. The most common primary tumors of the spleen are sarcomas, many of which have been linked to various environmental and occu-pational exposures such as vinyl chloride or thorium dioxide. Isolated splenic metastases are extremely unusual but may occur in the setting of a concomitant carcinoma.81 Lung carcinoma is the tumor that most commonly spreads to the spleen, although, colorectal, ovarian, and melanoma may also metastasize to the spleen.82,83 If after a | Surgery_Schwartz. Response to splenec-tomy was 76% overall at 1 year; overall complication rate was 28%, including 21 perioperative deaths.80 Thrombosis, hemor-rhage, and infection complications were common, with preop-erative thrombocytopenia an independent predictor of mortality risk. These data underscore the severity of this malignancy and emphasize the need for careful patient selection when consider-ing splenectomy in AMM.79Tumors and Metastasis. Primary tumors of the spleen are typ-ically benign or malignant variants of vascular neoplasms. The most common primary tumors of the spleen are sarcomas, many of which have been linked to various environmental and occu-pational exposures such as vinyl chloride or thorium dioxide. Isolated splenic metastases are extremely unusual but may occur in the setting of a concomitant carcinoma.81 Lung carcinoma is the tumor that most commonly spreads to the spleen, although, colorectal, ovarian, and melanoma may also metastasize to the spleen.82,83 If after a |
Surgery_Schwartz_10087 | Surgery_Schwartz | of a concomitant carcinoma.81 Lung carcinoma is the tumor that most commonly spreads to the spleen, although, colorectal, ovarian, and melanoma may also metastasize to the spleen.82,83 If after a thorough examination, an isolated splenic metastasis is confirmed, a laparoscopic splenectomy with intact spleen retrieval may be considered.81-83Miscellaneous Disorders and LesionsInfections and Abscesses. Primary infections of the spleen although uncommon in immunocompetent adults, are particu-larly reported in the immunocompromised population or those with a history of intravenous recreational drug abuse.84,85 The potential effects of certain systemic infections on the spleen merit close attention, mostly because of the dreaded risk of spontaneous splenic rupture. Infectious mononucleosis due to Brunicardi_Ch34_p1517-p1548.indd 152923/02/19 2:36 PM 1530SPECIFIC CONSIDERATIONSPART IIeither Epstein-Barr virus or cytomegalovirus infection imparts a small but often-discussed risk of | Surgery_Schwartz. of a concomitant carcinoma.81 Lung carcinoma is the tumor that most commonly spreads to the spleen, although, colorectal, ovarian, and melanoma may also metastasize to the spleen.82,83 If after a thorough examination, an isolated splenic metastasis is confirmed, a laparoscopic splenectomy with intact spleen retrieval may be considered.81-83Miscellaneous Disorders and LesionsInfections and Abscesses. Primary infections of the spleen although uncommon in immunocompetent adults, are particu-larly reported in the immunocompromised population or those with a history of intravenous recreational drug abuse.84,85 The potential effects of certain systemic infections on the spleen merit close attention, mostly because of the dreaded risk of spontaneous splenic rupture. Infectious mononucleosis due to Brunicardi_Ch34_p1517-p1548.indd 152923/02/19 2:36 PM 1530SPECIFIC CONSIDERATIONSPART IIeither Epstein-Barr virus or cytomegalovirus infection imparts a small but often-discussed risk of |
Surgery_Schwartz_10088 | Surgery_Schwartz | due to Brunicardi_Ch34_p1517-p1548.indd 152923/02/19 2:36 PM 1530SPECIFIC CONSIDERATIONSPART IIeither Epstein-Barr virus or cytomegalovirus infection imparts a small but often-discussed risk of spontaneous splenic rup-ture in both adults and children. The true incidence may be underreported, however. Recent case reports abound in the lit-erature regarding spontaneous splenic rupture due to a variety of infectious causes (malaria, Listeria infection, fungal infec-tions, dengue, and Q fever, to name a few) as well as a vari-ety of neoplastic and other noninfectious causes (lymphoma, angiosarcoma, amyloidosis, pregnancy). The presumed patho-physiologic mechanism is infiltration of the splenic paren-chyma with inflammatory cells, which distorts the architecture and fibrous support system of the spleen and thins the splenic capsule.86 In this setting, splenic rupture can occur spontane-ously or after a seemingly minor external trauma or even a Valsalva maneuver.Abscesses of the spleen | Surgery_Schwartz. due to Brunicardi_Ch34_p1517-p1548.indd 152923/02/19 2:36 PM 1530SPECIFIC CONSIDERATIONSPART IIeither Epstein-Barr virus or cytomegalovirus infection imparts a small but often-discussed risk of spontaneous splenic rup-ture in both adults and children. The true incidence may be underreported, however. Recent case reports abound in the lit-erature regarding spontaneous splenic rupture due to a variety of infectious causes (malaria, Listeria infection, fungal infec-tions, dengue, and Q fever, to name a few) as well as a vari-ety of neoplastic and other noninfectious causes (lymphoma, angiosarcoma, amyloidosis, pregnancy). The presumed patho-physiologic mechanism is infiltration of the splenic paren-chyma with inflammatory cells, which distorts the architecture and fibrous support system of the spleen and thins the splenic capsule.86 In this setting, splenic rupture can occur spontane-ously or after a seemingly minor external trauma or even a Valsalva maneuver.Abscesses of the spleen |
Surgery_Schwartz_10089 | Surgery_Schwartz | of the spleen and thins the splenic capsule.86 In this setting, splenic rupture can occur spontane-ously or after a seemingly minor external trauma or even a Valsalva maneuver.Abscesses of the spleen are uncommon, with an incidence reported to be 0.14% to 0.7%.87-89 They occur more frequently in tropical locations, where they are associated with throm-bosed splenic vessels and infarction in patients with sickle cell anemia. Five distinct mechanisms of splenic abscess formation have been described: (a) hematogenous infection; (b) contigu-ous infection; (c) hemoglobinopathy; (d) immunosuppression, including HIV infection and chemotherapy; and (e) trauma. The presentation of splenic abscess frequently is delayed, with most patients enduring symptoms for 16 to 22 days before diagno-sis. Clinical manifestations include fever, left upper quadrant pain, leukocytosis, and splenomegaly in about one-third of patients. The diagnosis is confirmed by ultrasound or CT scan, which has a 95% | Surgery_Schwartz. of the spleen and thins the splenic capsule.86 In this setting, splenic rupture can occur spontane-ously or after a seemingly minor external trauma or even a Valsalva maneuver.Abscesses of the spleen are uncommon, with an incidence reported to be 0.14% to 0.7%.87-89 They occur more frequently in tropical locations, where they are associated with throm-bosed splenic vessels and infarction in patients with sickle cell anemia. Five distinct mechanisms of splenic abscess formation have been described: (a) hematogenous infection; (b) contigu-ous infection; (c) hemoglobinopathy; (d) immunosuppression, including HIV infection and chemotherapy; and (e) trauma. The presentation of splenic abscess frequently is delayed, with most patients enduring symptoms for 16 to 22 days before diagno-sis. Clinical manifestations include fever, left upper quadrant pain, leukocytosis, and splenomegaly in about one-third of patients. The diagnosis is confirmed by ultrasound or CT scan, which has a 95% |
Surgery_Schwartz_10090 | Surgery_Schwartz | Clinical manifestations include fever, left upper quadrant pain, leukocytosis, and splenomegaly in about one-third of patients. The diagnosis is confirmed by ultrasound or CT scan, which has a 95% sensitivity and specificity. Common organ-isms are aerobic microbes (streptococci and Escherichia coli), but other microorganisms have also been isolated (Mycobac-terium tuberculosis and Salmonella typhi). Upon discovery of a splenic abscess, broad-spectrum antibiotics should be initi-ated, with adjustment to more specific therapy based on culture results and continuation of treatment for a minimum of 14 days. Splenectomy is the operation of choice, but percutaneous and open drainage are options for patients who either cannot toler-ate splenectomy or where the clinical scenario warrants splenic preservation, particularly in children.90 Percutaneous drainage is often successful for patients with unilocular disease and may result in avoidance of splenectomy. Patients with multilocular disease | Surgery_Schwartz. Clinical manifestations include fever, left upper quadrant pain, leukocytosis, and splenomegaly in about one-third of patients. The diagnosis is confirmed by ultrasound or CT scan, which has a 95% sensitivity and specificity. Common organ-isms are aerobic microbes (streptococci and Escherichia coli), but other microorganisms have also been isolated (Mycobac-terium tuberculosis and Salmonella typhi). Upon discovery of a splenic abscess, broad-spectrum antibiotics should be initi-ated, with adjustment to more specific therapy based on culture results and continuation of treatment for a minimum of 14 days. Splenectomy is the operation of choice, but percutaneous and open drainage are options for patients who either cannot toler-ate splenectomy or where the clinical scenario warrants splenic preservation, particularly in children.90 Percutaneous drainage is often successful for patients with unilocular disease and may result in avoidance of splenectomy. Patients with multilocular disease |
Surgery_Schwartz_10091 | Surgery_Schwartz | preservation, particularly in children.90 Percutaneous drainage is often successful for patients with unilocular disease and may result in avoidance of splenectomy. Patients with multilocular disease will often require multiple drains and therefore often benefit from total splenectomy.Cysts. Splenic cysts (Fig. 34-5) can be categorized according to a number of criteria; one clinically relevant scheme is to char-acterize true splenic cysts as either parasitic or nonparasitic.86Parasitic Infections. Parasitic infection is the most common cause of splenic cysts worldwide, and the majority are due to Echinococcus species. These cysts are more commonly found in areas where the pathogen is endemic. Symptoms, when present, generally are related to the presence of a mass lesion in the left upper quadrant or a lesion that impinges on the stomach. Ultra-sound can establish the presence of a cystic lesion and occasion-ally incidentally detect asymptomatic lesions as well. Serologic testing for | Surgery_Schwartz. preservation, particularly in children.90 Percutaneous drainage is often successful for patients with unilocular disease and may result in avoidance of splenectomy. Patients with multilocular disease will often require multiple drains and therefore often benefit from total splenectomy.Cysts. Splenic cysts (Fig. 34-5) can be categorized according to a number of criteria; one clinically relevant scheme is to char-acterize true splenic cysts as either parasitic or nonparasitic.86Parasitic Infections. Parasitic infection is the most common cause of splenic cysts worldwide, and the majority are due to Echinococcus species. These cysts are more commonly found in areas where the pathogen is endemic. Symptoms, when present, generally are related to the presence of a mass lesion in the left upper quadrant or a lesion that impinges on the stomach. Ultra-sound can establish the presence of a cystic lesion and occasion-ally incidentally detect asymptomatic lesions as well. Serologic testing for |
Surgery_Schwartz_10092 | Surgery_Schwartz | quadrant or a lesion that impinges on the stomach. Ultra-sound can establish the presence of a cystic lesion and occasion-ally incidentally detect asymptomatic lesions as well. Serologic testing for echinococcal antibodies can confirm or exclude the cystic lesion as parasitic, an important piece of informa-tion when planning operative therapy. Symptomatic parasitic cysts are best treated with splenectomy. Avoidance of spillage of parasitic cyst contents into the peritoneal cavity to avoid the possibility of anaphylactic shock is an important principle in surgical management despite its rare occurrence.91Cysts resulting from trauma are termed pseudocysts due to their lack of an epithelial cell lining. Less common examples of nonparasitic cysts are dermoid, epidermoid, and epithelial cysts.92 The treatment of nonparasitic cysts depends on whether or not they produce symptoms. Asymptomatic nonparasitic cysts may be observed with close follow-up by ultrasound to exclude significant growth | Surgery_Schwartz. quadrant or a lesion that impinges on the stomach. Ultra-sound can establish the presence of a cystic lesion and occasion-ally incidentally detect asymptomatic lesions as well. Serologic testing for echinococcal antibodies can confirm or exclude the cystic lesion as parasitic, an important piece of informa-tion when planning operative therapy. Symptomatic parasitic cysts are best treated with splenectomy. Avoidance of spillage of parasitic cyst contents into the peritoneal cavity to avoid the possibility of anaphylactic shock is an important principle in surgical management despite its rare occurrence.91Cysts resulting from trauma are termed pseudocysts due to their lack of an epithelial cell lining. Less common examples of nonparasitic cysts are dermoid, epidermoid, and epithelial cysts.92 The treatment of nonparasitic cysts depends on whether or not they produce symptoms. Asymptomatic nonparasitic cysts may be observed with close follow-up by ultrasound to exclude significant growth |
Surgery_Schwartz_10093 | Surgery_Schwartz | The treatment of nonparasitic cysts depends on whether or not they produce symptoms. Asymptomatic nonparasitic cysts may be observed with close follow-up by ultrasound to exclude significant growth or expansion. Patients should be advised of the risk of cyst rupture with even minor abdominal trauma if they elect nonoperative management for large cysts. Small symptomatic nonparasitic cysts may be excised with splenic preservation, and large symptomatic nonparasitic cysts may be unroofed. Both of these operations may be performed laparoscopically.86Storage Diseases and Infiltrative Disorders Gaucher’s Disease Gaucher’s disease is an inherited lipid stor-age disorder characterized by the deposition of glucocerebroside in cells of the macrophage-monocyte system. The underlying abnormality is a deficiency in the activity of a lysosomal hydro-lase. Abnormal glycolipid storage results in organomegaly, particularly hepatomegaly and splenomegaly.93,94 Patients with Gaucher’s disease frequently | Surgery_Schwartz. The treatment of nonparasitic cysts depends on whether or not they produce symptoms. Asymptomatic nonparasitic cysts may be observed with close follow-up by ultrasound to exclude significant growth or expansion. Patients should be advised of the risk of cyst rupture with even minor abdominal trauma if they elect nonoperative management for large cysts. Small symptomatic nonparasitic cysts may be excised with splenic preservation, and large symptomatic nonparasitic cysts may be unroofed. Both of these operations may be performed laparoscopically.86Storage Diseases and Infiltrative Disorders Gaucher’s Disease Gaucher’s disease is an inherited lipid stor-age disorder characterized by the deposition of glucocerebroside in cells of the macrophage-monocyte system. The underlying abnormality is a deficiency in the activity of a lysosomal hydro-lase. Abnormal glycolipid storage results in organomegaly, particularly hepatomegaly and splenomegaly.93,94 Patients with Gaucher’s disease frequently |
Surgery_Schwartz_10094 | Surgery_Schwartz | deficiency in the activity of a lysosomal hydro-lase. Abnormal glycolipid storage results in organomegaly, particularly hepatomegaly and splenomegaly.93,94 Patients with Gaucher’s disease frequently experience symptoms related to splenomegaly, including early satiety and abdominal discom-fort, and to hypersplenism, including thrombocytopenia, normo-cytic anemia, and mild leukopenia. These latter findings occur as a result of excessive sequestration of formed blood elements in the spleen. Other symptoms in patients with Gaucher’s dis-ease include bone pain, pathologic fractures, and jaundice. Splenectomy may alleviate hematologic abnormalities in these patients with hypersplenism, but it does not correct the underly-ing disease process.95Niemann-Pick Disease Niemann-Pick disease is an inherited disorder of abnormal lysosomal storage of sphingomyelin and cholesterol in cells of the macrophage-monocyte system. Four types of the disease (A, B, C, and D) exist, with unique clinical | Surgery_Schwartz. deficiency in the activity of a lysosomal hydro-lase. Abnormal glycolipid storage results in organomegaly, particularly hepatomegaly and splenomegaly.93,94 Patients with Gaucher’s disease frequently experience symptoms related to splenomegaly, including early satiety and abdominal discom-fort, and to hypersplenism, including thrombocytopenia, normo-cytic anemia, and mild leukopenia. These latter findings occur as a result of excessive sequestration of formed blood elements in the spleen. Other symptoms in patients with Gaucher’s dis-ease include bone pain, pathologic fractures, and jaundice. Splenectomy may alleviate hematologic abnormalities in these patients with hypersplenism, but it does not correct the underly-ing disease process.95Niemann-Pick Disease Niemann-Pick disease is an inherited disorder of abnormal lysosomal storage of sphingomyelin and cholesterol in cells of the macrophage-monocyte system. Four types of the disease (A, B, C, and D) exist, with unique clinical |
Surgery_Schwartz_10095 | Surgery_Schwartz | an inherited disorder of abnormal lysosomal storage of sphingomyelin and cholesterol in cells of the macrophage-monocyte system. Four types of the disease (A, B, C, and D) exist, with unique clinical presentations. Types A and B result from a deficiency in lyso-somal hydrolase and are the forms most likely to demonstrate splenomegaly with its concomitant symptoms. Symptoms of splenomegaly are relieved by splenectomy.Amyloidosis Amyloidosis is a disorder of abnormal extra-cellular protein deposition and protein conformation disorder associated with a clonal plasma cell dyscrasia. There are mul-tiple forms of amyloidosis, each with its own individual clini-cal presentation, and the severity of disease may range from asymptomatic to multiorgan failure. Patients with primary amy-loidosis, associated with plasma cell dyscrasia, have splenic involvement in approximately 5% of cases. Secondary amy-loidosis, associated with chronic inflammatory conditions, also may present with an enlarged | Surgery_Schwartz. an inherited disorder of abnormal lysosomal storage of sphingomyelin and cholesterol in cells of the macrophage-monocyte system. Four types of the disease (A, B, C, and D) exist, with unique clinical presentations. Types A and B result from a deficiency in lyso-somal hydrolase and are the forms most likely to demonstrate splenomegaly with its concomitant symptoms. Symptoms of splenomegaly are relieved by splenectomy.Amyloidosis Amyloidosis is a disorder of abnormal extra-cellular protein deposition and protein conformation disorder associated with a clonal plasma cell dyscrasia. There are mul-tiple forms of amyloidosis, each with its own individual clini-cal presentation, and the severity of disease may range from asymptomatic to multiorgan failure. Patients with primary amy-loidosis, associated with plasma cell dyscrasia, have splenic involvement in approximately 5% of cases. Secondary amy-loidosis, associated with chronic inflammatory conditions, also may present with an enlarged |
Surgery_Schwartz_10096 | Surgery_Schwartz | with plasma cell dyscrasia, have splenic involvement in approximately 5% of cases. Secondary amy-loidosis, associated with chronic inflammatory conditions, also may present with an enlarged spleen and even spontaneous rup-ture.96 Symptoms of splenomegaly are relieved by splenectomy.Sarcoidosis Sarcoidosis is an inflammatory disease of young adults characterized by noncaseating granulomas in affected tissues. Signs and symptoms of the disease range in severity and typically are nonspecific, such as fatigue and malaise. Any organ system may be involved. The most commonly involved organ is the lung, followed by the spleen. Splenomegaly occurs in approximately 25% of patients. Massive splenomegaly (>1 kg) is rare with a reported incidence of 3%.97,98 Other affected tissues include the lymph nodes, eyes, joints, liver, spleen, and heart. When splenomegaly occurs and causes symp-toms related to size or hypersplenism, splenectomy effectively relieves symptoms and corrects hematologic | Surgery_Schwartz. with plasma cell dyscrasia, have splenic involvement in approximately 5% of cases. Secondary amy-loidosis, associated with chronic inflammatory conditions, also may present with an enlarged spleen and even spontaneous rup-ture.96 Symptoms of splenomegaly are relieved by splenectomy.Sarcoidosis Sarcoidosis is an inflammatory disease of young adults characterized by noncaseating granulomas in affected tissues. Signs and symptoms of the disease range in severity and typically are nonspecific, such as fatigue and malaise. Any organ system may be involved. The most commonly involved organ is the lung, followed by the spleen. Splenomegaly occurs in approximately 25% of patients. Massive splenomegaly (>1 kg) is rare with a reported incidence of 3%.97,98 Other affected tissues include the lymph nodes, eyes, joints, liver, spleen, and heart. When splenomegaly occurs and causes symp-toms related to size or hypersplenism, splenectomy effectively relieves symptoms and corrects hematologic |
Surgery_Schwartz_10097 | Surgery_Schwartz | lymph nodes, eyes, joints, liver, spleen, and heart. When splenomegaly occurs and causes symp-toms related to size or hypersplenism, splenectomy effectively relieves symptoms and corrects hematologic abnormalities such Brunicardi_Ch34_p1517-p1548.indd 153023/02/19 2:36 PM 1531THE SPLEENCHAPTER 34as anemia and thrombocytopenia. Spontaneous splenic rupture has been reported in sarcoidosis.94Splenic Artery Aneurysm Although splenic artery aneurysm is rare, it is the most common visceral artery aneurysm. Women are four times more likely to be affected than men. The aneu-rysm usually arises in the middle to distal portion of the splenic artery.99,100 Indications for treatment include presence of symp-toms, pregnancy, intention to become pregnant, and presence of pseudoaneurysms associated with inflammatory processes. Aneurysm resection or ligation alone is acceptable for amenable lesions in the mid-splenic artery, but distal lesions in close prox-imity to the splenic hilum should be | Surgery_Schwartz. lymph nodes, eyes, joints, liver, spleen, and heart. When splenomegaly occurs and causes symp-toms related to size or hypersplenism, splenectomy effectively relieves symptoms and corrects hematologic abnormalities such Brunicardi_Ch34_p1517-p1548.indd 153023/02/19 2:36 PM 1531THE SPLEENCHAPTER 34as anemia and thrombocytopenia. Spontaneous splenic rupture has been reported in sarcoidosis.94Splenic Artery Aneurysm Although splenic artery aneurysm is rare, it is the most common visceral artery aneurysm. Women are four times more likely to be affected than men. The aneu-rysm usually arises in the middle to distal portion of the splenic artery.99,100 Indications for treatment include presence of symp-toms, pregnancy, intention to become pregnant, and presence of pseudoaneurysms associated with inflammatory processes. Aneurysm resection or ligation alone is acceptable for amenable lesions in the mid-splenic artery, but distal lesions in close prox-imity to the splenic hilum should be |
Surgery_Schwartz_10098 | Surgery_Schwartz | with inflammatory processes. Aneurysm resection or ligation alone is acceptable for amenable lesions in the mid-splenic artery, but distal lesions in close prox-imity to the splenic hilum should be treated with concomitant splenectomy. An excellent prognosis follows elective treatment. Splenic artery embolization has been used to treat splenic artery aneurysm; however, this may result in painful splenic infarction or abscess.Portal Hypertension Portal hypertension can result from numerous causes but is usually due to liver cirrhosis. Sple-nomegaly and splenic congestion often accompany portal hypertension, which leads to sequestration and destruction of circulating cells in the spleen. Splenectomy is not indicated for hypersplenism per se in patients with portal hypertension as there is no correlation between the degree of pancytopenia and long-term survival in these patients.7 In rare circumstances in which splenectomy is required to reduce bleeding from esopha-geal varices | Surgery_Schwartz. with inflammatory processes. Aneurysm resection or ligation alone is acceptable for amenable lesions in the mid-splenic artery, but distal lesions in close prox-imity to the splenic hilum should be treated with concomitant splenectomy. An excellent prognosis follows elective treatment. Splenic artery embolization has been used to treat splenic artery aneurysm; however, this may result in painful splenic infarction or abscess.Portal Hypertension Portal hypertension can result from numerous causes but is usually due to liver cirrhosis. Sple-nomegaly and splenic congestion often accompany portal hypertension, which leads to sequestration and destruction of circulating cells in the spleen. Splenectomy is not indicated for hypersplenism per se in patients with portal hypertension as there is no correlation between the degree of pancytopenia and long-term survival in these patients.7 In rare circumstances in which splenectomy is required to reduce bleeding from esopha-geal varices |
Surgery_Schwartz_10099 | Surgery_Schwartz | is no correlation between the degree of pancytopenia and long-term survival in these patients.7 In rare circumstances in which splenectomy is required to reduce bleeding from esopha-geal varices exacerbated by thrombocytopenia, a concomitant splenorenal shunt procedure may be performed to decompress the portal system.Sinistral hypertension secondary to splenic vein thrombo-sis, on the other hand, is potentially curable with splenectomy. Patients that are bleeding from isolated gastric varices who have normal liver function test results, especially those with a pre-vious history of pancreatitis, should be examined for splenic vein thrombosis and treated with splenectomy if findings are positive.Idiopathic portal hypertension (IPH) is extremely rare, and it is characterized by the absence of cirrhosis or other clear etiologies such as hepatic or portal vein thrombosis, cardiac failure, or hematologic disorders.101-104 Hypersplen-ism often develops in these patients due to hyperactivity | Surgery_Schwartz. is no correlation between the degree of pancytopenia and long-term survival in these patients.7 In rare circumstances in which splenectomy is required to reduce bleeding from esopha-geal varices exacerbated by thrombocytopenia, a concomitant splenorenal shunt procedure may be performed to decompress the portal system.Sinistral hypertension secondary to splenic vein thrombo-sis, on the other hand, is potentially curable with splenectomy. Patients that are bleeding from isolated gastric varices who have normal liver function test results, especially those with a pre-vious history of pancreatitis, should be examined for splenic vein thrombosis and treated with splenectomy if findings are positive.Idiopathic portal hypertension (IPH) is extremely rare, and it is characterized by the absence of cirrhosis or other clear etiologies such as hepatic or portal vein thrombosis, cardiac failure, or hematologic disorders.101-104 Hypersplen-ism often develops in these patients due to hyperactivity |
Surgery_Schwartz_10100 | Surgery_Schwartz | cirrhosis or other clear etiologies such as hepatic or portal vein thrombosis, cardiac failure, or hematologic disorders.101-104 Hypersplen-ism often develops in these patients due to hyperactivity of the spleen; defined as a triad of splenomegaly, pancytopenia, 6ACBDFigure 34-5. A. Computed tomography (CT) scan of giant splenic cyst. B. Three-dimensional CT reconstruction of splenic cyst. C and D. Macroscopic aspect of a multicystic spleen lesion.Brunicardi_Ch34_p1517-p1548.indd 153123/02/19 2:37 PM 1532SPECIFIC CONSIDERATIONSPART IIand normocellularity of bone marrow warranting intervention by way of splenic artery embolization or splenectomy. Par-tial splenic embolization (PSE) has recently been utilized with good success in prohibitively high operative risk patients with severe hypersplenism.105Felty’s Syndrome The triad of rheumatoid arthritis, spleno-megaly, and neutropenia is called Felty’s syndrome. It exists in approximately 3% of all patients with rheumatoid arthritis, | Surgery_Schwartz. cirrhosis or other clear etiologies such as hepatic or portal vein thrombosis, cardiac failure, or hematologic disorders.101-104 Hypersplen-ism often develops in these patients due to hyperactivity of the spleen; defined as a triad of splenomegaly, pancytopenia, 6ACBDFigure 34-5. A. Computed tomography (CT) scan of giant splenic cyst. B. Three-dimensional CT reconstruction of splenic cyst. C and D. Macroscopic aspect of a multicystic spleen lesion.Brunicardi_Ch34_p1517-p1548.indd 153123/02/19 2:37 PM 1532SPECIFIC CONSIDERATIONSPART IIand normocellularity of bone marrow warranting intervention by way of splenic artery embolization or splenectomy. Par-tial splenic embolization (PSE) has recently been utilized with good success in prohibitively high operative risk patients with severe hypersplenism.105Felty’s Syndrome The triad of rheumatoid arthritis, spleno-megaly, and neutropenia is called Felty’s syndrome. It exists in approximately 3% of all patients with rheumatoid arthritis, |
Surgery_Schwartz_10101 | Surgery_Schwartz | Syndrome The triad of rheumatoid arthritis, spleno-megaly, and neutropenia is called Felty’s syndrome. It exists in approximately 3% of all patients with rheumatoid arthritis, two-thirds of whom are women. Immune complexes coat the surface of white blood cells, which leads to their sequestration and clear-ance in the spleen with subsequent neutropenia. This neutro-penia (<2000 neutrophils/mm3) increases the risk for recurrent infections and often drives the decision for splenectomy. The size of the spleen is variable, from nonpalpable in 5% to 10% of patients to massively enlarged upwards of 4 times heavier than normal in others. Corticosteroids, hematopoietic growth factors, methotrexate, and splenectomy have all been used to treat the neutropenia of Felty’s syndrome. More recently, ritux-imab has been tried as a second line agent in refractory Felty’s syndrome.106,107Overall response to splenectomy is excellent, with >80% of patients showing a durable increase in white blood cell | Surgery_Schwartz. Syndrome The triad of rheumatoid arthritis, spleno-megaly, and neutropenia is called Felty’s syndrome. It exists in approximately 3% of all patients with rheumatoid arthritis, two-thirds of whom are women. Immune complexes coat the surface of white blood cells, which leads to their sequestration and clear-ance in the spleen with subsequent neutropenia. This neutro-penia (<2000 neutrophils/mm3) increases the risk for recurrent infections and often drives the decision for splenectomy. The size of the spleen is variable, from nonpalpable in 5% to 10% of patients to massively enlarged upwards of 4 times heavier than normal in others. Corticosteroids, hematopoietic growth factors, methotrexate, and splenectomy have all been used to treat the neutropenia of Felty’s syndrome. More recently, ritux-imab has been tried as a second line agent in refractory Felty’s syndrome.106,107Overall response to splenectomy is excellent, with >80% of patients showing a durable increase in white blood cell |
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