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The white pulp (lymphoid tissue) functions in antigen processing and antibody production.
The red pulp (monocyte-macrophage system) serves as a filter, retaining defective 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.
It is usually associated with splenomegaly.
It causes cytopenias with associated compensatory bone marrow hyperplasia.
It usually is corrected by splenectomy, if indicated.
Table 26–1 lists the causes of hypersplenism. Table 26–2 lists the causes of massive splenomegaly.
TABLE 26–1CLASSIFICATION AND THE MOST COMMON CAUSES OF SPLENOMEGALY AND HYPERSPLENISM
Right-sided congestive heart failure
Budd-Chiari syndrome (hepatic vein thrombosis with or without inferior vena cava extension)
Cirrhosis with portal hypertension
Portal or splenic vein thrombosis
Acute HIV infection/chronic infection
Rubella (rare except newborns)
Cytomegalovirus infection (rare except newborns)
Herpes simplex (rare except newborns)
Subacute bacterial endocarditis
Anaplasmosis (formerly ehrlichiosis)
Rickettsial diseases (scrub typhus, Rocky Mountain spotted fever, Q fever)
Splenic abscess (most common organisms are Enterobacteriaceae, Staphylococcus aureus, streptococcus group D, and anaerobic organisms as part of mixed flora infections)
Systemic candidiasis and hepatosplenic candidiasis
Trypanosomiasis (cruzi, brucei)
Toxoplasmosis (rare except newborns)
Visceral larva migrans (Toxocara infection)
Systemic lupus erythematosus (SLE)
Juvenile rheumatoid arthritis
Autoimmune lymphoproliferative syndrome (ALP syndrome)
Common variable immunodeficiency
Anti-D immunoglobulin administration
Associated with hemolysis
Thalassemia major and intermedia
Pyruvate kinase deficiency
Autoimmune hemolytic anemia (rare)
Sickle cell disease, more common in early childhood (splenic sequestration), hemoglobin C disease, and some other hemoglobinopathies
Splenic hematoma (splenic cysts are usually a late complication of a hematoma)
Littoral cell angioma
Disorders of sphingolipid metabolism
Amyloidosis (light-chain amyloid and amyloid A protein)
Multicentric Castleman disease
Chronic lymphocytic leukemia (especially prolymphocytic variant)
Chronic myeloid leukemia
Hairy cell leukemia
Heavy chain disease
Acute leukemia (acute lymphoblastic leukemia/acute myeloid leukemia)
Hodgkin and other lymphomas
Metastatic carcinoma (rare)
Malignant fibrous histiocytoma
Granulocyte colony-stimulating factor administration
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