Common Causes of Splenomegaly

Causes

Examples

Chronic hemolytic anemia

Hemoglobinopathies, including the thalassemias, sickle cell hemoglobin variants (eg, hemoglobin S-C disease, sickle/beta thalassemia), and congenital Heinz body hemolytic anemias

Red blood cell enzymopathies (eg, pyruvate kinase deficiency)

Red blood cell shape abnormalities (eg, hereditary spherocytosis, hereditary elliptocytosis)

Congestion

Certain vascular malformations or thrombosis of the portal venous vasculature

Cirrhosis with portal hypertension

External compression of portal or splenic veins

Infection or inflammation

Acute infections (eg, infectious hepatitis, infectious mononucleosis, psittacosis, subacute bacterial endocarditis)

Chronic infections (eg, bartonellosis, brucellosis, candidiasis, histoplasmosis, malaria, miliary tuberculosis, visceral leishmaniasis [kala-azar], syphilis)

Systemic rheumatic disorders (eg, rheumatoid arthritis with Felty syndrome), systemic lupus erythematosus

Hemophagocytic lymphohistiocytosis

Langerhans cell histiocytosis

Sarcoidosis

Secondary amyloidosis

Myeloproliferative and lymphoproliferative disorders

Leukemias, especially chronic lymphocytic, large granular lymphocytic, and chronic myelogenous leukemia

Lymphomas, especially hairy cell leukemia and splenic marginal zone lymphoma

Primary myelofibrosis

Polycythemia vera

Storage diseases

Gaucher disease

Niemann-Pick disease

Wolman disease

Structural

Splenic cysts, usually caused by resolution of previous intrasplenic hematoma

Adapted from Williams WJ, et al: Hematology. New York, McGraw-Hill Book Company, 1976.

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