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Table 46-2

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