Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content ++ THE SPLEEN ++ The white pulp (lymphoid tissue) functions in antigen processing and antibody production. The red pulp (monocyte-macrophage system) serves as a filter, retaining defective or effete blood cells and foreign particles. ++ HYPERSPLENISM (INCREASED SPLENIC FUNCTION) ++ Hypersplenism is considered "appropriate" if it is an exaggeration of normal function, as in hereditary spherocytosis or idiopathic thrombocytopenic purpura, or "inappropriate" if the hyperfunction is a result of vascular congestion or infiltrative disease. Usually associated with splenomegaly. Causes cytopenias with associated compensatory bone marrow hyperplasia. Usually corrected by splenectomy, if indicated. Table 28–1 lists the causes of hypersplenism; Table 28–2 lists the causes of massive splenomegaly. ++Table Graphic Jump LocationTABLE 28–1CLASSIFICATION AND THE MOST COMMON CAUSES OF SPLENOMEGALY AND HYPERSPLENISMView Table|Favorite Table|Download (.pdf) TABLE 28–1 CLASSIFICATION AND THE MOST COMMON CAUSES OF SPLENOMEGALY AND HYPERSPLENISM Congestive Right-sided congestive heart failure Budd-Chiari syndrome (inferior vena cava and hepatic vein thrombosis) Cirrhosis with portal hypertension Portal or splenic vein thrombosis Immunologic Viral infection Acute HIV infection/chronic infection Acute mononucleosis Dengue fever Rubella (rare except newborns) Cytomegalovirus infection (rare except newborns) Herpes simplex (rare except newborns) Bacterial infection Subacute bacterial endocarditis Brucellosis Tularemia Melioidosis Listeriosis Plague Secondary syphilis Relapsing fever Psittacosis Ehrlichiosis Rickettsial diseases (scrub typhus, Rocky Mountain spotted fever, Q fever) Tuberculosis Splenic abscess (most common organisms are Enterobacteriaceae, Staphylococcus aureus, streptococcus group D, and anaerobic organisms as part of mixed flora infections) Fungal Infection Blastomycosis Histoplasmosis Systemic candidiasis and hepatosplenic candidiasis Parasitic infection Malaria Kala-azar Leishmaniasis Schistosomiasis Babesiosis Coccidioidomycosis Paracoccidioidomycosis Trypanosomiasis (cruzi, brucei) Toxoplasmosis (rare except newborns) Echinococcosis Cysticercosis Visceral larva migrans (Toxocara infection) Inflammatory/autoimmune Systemic lupus erythematosus (SLE) Felty syndrome Juvenile rheumatoid arthritis Autoimmune lymphoproliferative syndrome (ALP syndrome) Hemophagocytic syndrome Common variable immunodeficiency Anti-D immunoglobulin administration Associated with hemolysis Thalassemia major and intermedia Pyruvate kinase deficiency Hereditary spherocytosis Autoimmune hemolytic anemia (rare) Sickle cell disease, more common in early childhood (splenic sequestration), hemoglobin C disease, and some other hemoglobinopathies Infiltrative Nonmalignant Splenic hematoma (splenic cysts are usually a late complication of a hematoma) Littoral cell angioma Disorders of sphingolipid metabolism Gaucher disease Niemann-Pick disease Cystinosis Amyloidosis (light-chain amyloid and amyloid A protein) Multicentric Castleman disease Mastocytosis Hypereosinophilic syndrome Sarcoidosis Extramedullary hematopoiesis Primary myelofibrosis Osteopetrosis (childhood) Thalassemia major Malignant Hematologic Chronic lymphocytic leukemia (especially prolymphocytic variant) Chronic myeloid leukemia Polycythemia vera Hairy cell leukemia Heavy chain disease Hepatosplenic lymphoma Acute leukemia (acute lymphoblastic leukemia/acute myeloid leukemia) Hodgkin and other lymphomas Nonhematologic Metastatic carcinoma (rare) Neuroblastoma Wilms tumor Leiomyosarcoma Fibrosarcoma Malignant fibrous histiocytoma Kaposi sarcoma Hemangiosarcoma Lymphangiosarcoma Hemangioendothelial sarcoma Iatrogenic Granulocyte colony-stimulating factor administration Erythropoietin administration Source: Williams Hematology, 8th ed, Chap. 55, Table 55–1, p. 817. ++Table Graphic Jump LocationTABLE 28–2CAUSES OF MASSIVE SPLENOMEGALYView Table|Favorite Table|Download (.pdf) TABLE 28–2 CAUSES OF MASSIVE SPLENOMEGALY Myeloproliferative disorders Primary myelofibrosis Chronic myeloid leukemia Lymphomas Hairy cell leukemia Chronic lymphocytic leukemia (especially prolymphocytic ... GET ACCESS TO THIS RESOURCE Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth Get Free Access Through Your Institution Contact your institution's library to ask if they subscribe to McGraw-Hill Medical Products. What is MyAccess? Create a FREE MyAccess profile to: Use this site remotely Bookmark your favorite content Track your self-assessment progress and more!