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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.
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tieller Thrombopenia”. Wien Klin Wochenschr 29:1451, 1916.
5. Crosby WH: Hypersplenism. Annu Rev Med 13:127, 1962.
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9. Dear TN, Colledge WH, Carlton MB, et al: The Hox11 gene is essential for cell survival
during spleen development. Development 121:2909, 1995.
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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,
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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.
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