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868  Part VI:  The Erythrocyte                               Chapter 56:  Hypersplenism and Hyposplenism              869




                                                                        REFERENCES

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                                                                          2.  Chauffard AME: Des hepatites d’origine splenique. Semin Med 19:177, 1899.
                                                                          3.  Sutherland GA, Burghard FF: The treatment of splenic anaemia by splenectomy. Lancet
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                                                                          4.  Kaznelson P: Verschwinden der hamorrhagischen Diathesis bei einen falle von “Essen-
                                                                           tieller Thrombopenia”. Wien Klin Wochenschr 29:1451, 1916.
                                                                          5.  Crosby WH: Hypersplenism. Annu Rev Med 13:127, 1962.
                                                                          6.  Mutchnick MG, Lerner E, Conn HO: Effect of portacaval anastomosis on hyper-
                                                                           splenism. Dig Dis Sci 25:929, 1980.
                                                                          7.  Jabbour N, Zajko A, Orons P, et al: Does transjugular intrahepatic portosystemic shunt
                                                                           (TIPS) resolve thrombocytopenia associated with cirrhosis? Dig Dis Sci 43:2459, 1998.
                                                                          8.  Roberts CW, Shutter JR, Korsmeyer SJ: Hox11 controls the genesis of the spleen. Nature
                                                                           368:747, 1994.
                                                                          9.  Dear TN, Colledge WH, Carlton MB, et al: The Hox11 gene is essential for cell survival
                                                                           during spleen development. Development 121:2909, 1995.
                                                                          10.  Roberts CW, Sonder AM, Lumsden A, et al: Developmental expression of HOV11 and
                                                                           specification of splenic cell fate. Am J Pathol 146:1089, 1995.
                                                                          11.  Steininger B, Barth P, Herbst B, et al: The species-specific structure of microanatomical
                  Figure 56–3.  Blood film of splenectomized patient showing three red   compartments in the human spleen. Immunology 92:307, 1997.
                  cells with Howell-Jolly bodies (nuclear remnants). Note also the cluster     12.  Bourdessoule D, Gaulard P, Mason DY: Preferential localization of human lympho-
                  of acanthocytes and scattered spheroacanthocytes and the target cell.   cytes bearing −/− T cell receptors to the red pulp of the spleen. J Clin Pathol 43:461,
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                  These changes are also indicative of postsplenectomy red cell changes.     13.  Kraus MD: Splenic histology and histopathology: An update. Semin Diagn Pathol 20:84,
                  (Reproduced with permission from Lichtman’s Atlas of Hematology, www.   2003.
                  accessmedicine.com.)                                    14.  Rosse WF: The spleen as a filter [editorial]. N Engl J Med 317:704, 1987.
                                                                          15.  Bowdler AJ: Splenomegaly and hypersplenism. Clin Haematol 12:467, 1983.
                                                                          16.  Areas Elenas N, Ewald R, Crosby WH: The reservoir function of the spleen and its
                                                                           relation to postsplenectomy anemia of the dog. Blood 24:299, 1964.
                  hemoglobin level as the vesicles are removed (pitted) by the spleen.     17.  Wadenvik H, Kutti J: The spleen and pooling of blood cells. Eur J Haematol 41:1, 1988.
                  In asplenic individuals the vesicles are more numerous and enlarge,     18.  Aster RH: Pooling of platelets in the spleen: Role in the pathogenesis of “hypersplenic
                                                                           thrombocytopenia.” J Clin Invest 45:645, 1966.
                                                                    88
                  forming vacuoles that are evident by interference-phase microscopy.      19.  Gurakar A, Fagiuoli S, Gavaler JS, et al: The use of granulocyte-macrophage colony-
                  This finding is the most specific evidence of hyposplenism, followed by   stimulating factor to enhance hematologic parameters of patients with cirrhosis and
                                                                           hypersplenism. J Hepatol 21:582, 1994.
                  the presence of DNA inclusions in circulating red cells (Howell-Jolly       20.  Karpatkin S: The spleen and thrombocytopenia. Clin Haematol 12:591, 1983.
                  bodies; Fig. 56–3).                                     21.  Zwiebel WJ, Mountford RA, Halliwell MJ, Wells PN: Splanchnic blood flow in patients
                     Oxidative drugs may produce Heinz bodies even in normal indi-  with cirrhosis and portal hypertension: Investigation with duplex Doppler US. Radiol-
                                                                           ogy 194:807, 1995.
                  viduals, but the spleen effectively removes these red cell inclusions, as     22.  Brubaker LH, Johnson CA: Correlation of splenomegaly and abnormal neutrophil
                  well as Pappenheimer bodies. Heinz bodies may be observed in supravi-  pooling (margination). J Lab Clin Med 92:508, 1978.
                  tally stained blood films after splenectomy. Nucleated red cells rarely are     23.  Siciliano M, Tomasello D, Milani A, et al: Reduced serum levels of immunoreactive ery-
                  seen on blood films after splenectomy, except in patients with hemolytic   thropoietin in patients with cirrhosis and chronic anemia. Hepatology 22:1132, 1995.
                  disorders in whom the number of nucleated red cells may increase dra-    24.  Vasilopoulos S, Hally R, Caro J, et al: Erythropoietin response to post-liver transplanta-
                                                                           tion anemia. Liver Transpl 6:349, 2000.
                  matically. The reticulocyte count remains within normal values, and the     25.  Hess CE, Ayers CR, Sandusky WR, et al: Mechanism of dilutional anemia in massive
                  life span of red cells is unchanged as other organs take up the function   splenomegaly. Blood 47:629, 1976.
                  of removing senescent erythrocytes.                     26.  Zhang B, Lewis SM: Splenic hematocrit and the splenic plasma pool. Br J Haematol
                                                                           66:97, 1987.
                     Technetium-99m sulfur-colloid particles are used for spleen scan-    27.  Jandl JH: The anemia of liver disease: Observations on its mechanism. J Clin Invest
                  ning, a reliable measure of the capacity of the spleen to clear particulate   34:390, 1955.
                  matter from the bloodstream. 91                         28.  Christensen BE: Quantitative determination of splenic red cell blood destruction in
                                                                           patients with splenomegaly. Scand J Haematol 14:295, 1975.
                                                                          29.  McIntyre OR, Ebaugh FA: Palpable spleens in college freshmen. Ann Intern Med 66:301,
                                                                           1967.
                  THERAPY, COURSE, AND PROGNOSIS                          30.  Sty JR, Wells RG: Imaging the spleen,  in Disorders of the Spleen: Pathophysiology and
                  Vaccination against H. influenzae, N. meningitidis, and S. pneumoniae   Management, edited by C Pochedly, RH Sills, AD Schwartz, p 355. Marcel Dekker, New
                  is recommended in previously unvaccinated individuals prior to sple-  York, 1989.
                  nectomy.  Prophylactic immunization has significantly reduced, but     31.  Buehner M, Baker MS: The wandering spleen. Surg Gynecol Obstet 175:373, 1992.
                        92
                                                                          32.  Joyce RA, Boggs DR, Hasiba U, Srodes CH: Marginal neutrophil in the pool size in
                  not eliminated, the risk of overwhelming infection. 87,93–95  Oral penicillin   normal subjects as measured by epinephrine infusion. J Lab Clin Med 88:614, 1976.
                  or a macrolide antibiotic as prophylaxis for asplenic patients is recom-    33.  Alvarez OA, Lopera GA, Patel V, et al: Improvement of thrombocytopenia due to
                  mended based on publicized guidelines despite problems with compli-  hypersplenism after transjugular intrahepatic portosystemic shunt placement in cir-
                                                                           rhotic patients. Am J Gastroenterol 91:134, 1996.
                  ance and resistance. 96,97  Physicians should advise all asplenic patients     34.  Peck-Radosavljevic M: Hypersplenism. Eur J Gastroenterol Hepatol 13:317, 2001.
                  that any febrile episode (>38°C) should be considered an emergency     35.  Bashour FN, Teran JC, Mullen KD: Prevalence of peripheral blood cytopenias (hyper-
                  requiring immediate medical attention. A febrile asplenic patient must   splenism) in patients with nonalcoholic chronic liver disease.  Am  J  Gastroenterol
                                                                           95:2936, 2000.
                  have blood and urine cultures drawn, followed by antibiotic treatment.     36.  Liangpunsakul S, Ulmer BJ, Chalasani N: Predictors and implications of severe hyper-
                  Patients should be given written information about asplenia and carry   splenism in patients with cirrhosis. Am J Med Sci 326:111, 2003.
                  a card or medical alert bracelet to alert health professionals of the risk     37.  Qamar A, Grace N, Groszmann R, et al: Incidence, prevalence and clinical significance
                  of overwhelming infection. 96,97  Dental work, especially tooth extraction,   of abnormal hematological indices in compensated cirrhosis. Clin Gastroenterol Hepa-
                                                                           tol 7:689, 2009.
                  should be preceded by broad-spectrum antibiotics, such as amoxicillin,     38.  Civardi G, Vallisa D, Berte R, et al: Ultrasound guided fine needle biopsy of the spleen:
                  if the patient is not taking prophylactic antibiotics. Patients should be   High clinical efficacy and low risk in a multicenter Italian study. Am J Hematol 67:93,
                                                                           2001.
                  educated about risks of travel, including risk of malaria infection or ani-    39.  Pochedly C, Sills RH, Schwartz A: Disorders of the Spleen: Pathophysiology and Manage-
                  mal bites, which could be deadly unless promptly treated. 96,97  ment. Marcel Dekker, New York, 1989.






          Kaushansky_chapter 56_p0863-0870.indd   869                                                                   9/17/15   3:05 PM
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