Diseases Associated with Splenomegaly Grouped by Pathogenic Mechanism
| Enlargement Due to Increased Demand for Splenic Function | |
Reticuloendothelial system hyperplasia (for removal of defective erythrocytes) Spherocytosis Early sickle cell anemia Ovalocytosis Thalassemia major Hemoglobinopathies Paroxysmal nocturnal hemoglobinuria Pernicious anemia Immune hyperplasia Response to infection (viral, bacterial, fungal, parasitic) Infectious mononucleosis AIDS Viral hepatitis Cytomegalovirus Subacute bacterial endocarditis Bacterial septicemia Congenital syphilis Splenic abscess Tuberculosis Histoplasmosis Malaria | Leishmaniasis Trypanosomiasis Ehrlichiosis Disordered immunoregulation Rheumatoid arthritis (Felty's syndrome) Systemic lupus erythematosus Collagen vascular diseases Serum sickness Immune hemolytic anemias Immune thrombocytopenias Immune neutropenias Drug reactions Angioimmunoblastic lymphadenopathy Sarcoidosis Thyrotoxicosis (benign lymphoid hypertrophy) Interleukin-2 therapy Extramedullary hematopoiesis Myelofibrosis Marrow damage by toxins, radiation, strontium Marrow infiltration by tumors, leukemias, Gaucher's disease |
| Enlargement Due to Abnormal Splenic or Portal Blood Flow | |
Cirrhosis Hepatic vein obstruction Portal vein obstruction, intrahepatic or extrahepatic Cavernous transformation of the portal vein Splenic vein obstruction | Splenic artery aneurysm Hepatic schistosomiasis Congestive heart failure Hepatic echinococcosis Portal hypertension (any cause including the above): “Banti's disease” |
| Infiltration of the Spleen | |
Intracellular or extracellular depositions Amyloidosis Gaucher's disease Niemann-Pick disease Tangier disease Hurler's syndrome and other mucopolysaccharidoses Hyperlipidemias Benign and malignant cellular infiltrations Leukemias (acute, chronic, lymphoid, myeloid, monocytic, hairy cell) Lymphomas | Hodgkin's lymphoma Myeloproliferative syndromes (e.g., polycythemia vera, essential thrombocytosis) Angiosarcomas Metastatic tumors (melanoma is most common) Eosinophilic granuloma Histiocytic and dendritic cell neoplasms (Histiocytosis X) Hamartomas Hemangiomas, fibromas, lymphangiomas Splenic cysts |
| Unknown Etiology | |
Idiopathic splenomegaly Berylliosis | Iron-deficiency anemia |