Diseases Associated with Splenomegaly Grouped by Pathogenic Mechanism1
| Enlargement Due to Increased Demand for Splenic Function | |
|---|---|
| Reticuloendothelial system hyperplasia (for removal of defective erythrocytes) | Leishmaniasis |
| Spherocytosis | Trypanosomiasis |
| Early sickle cell anemia | Ehrlichiosis |
| Ovalocytosis | Disordered immunoregulation |
| Thalassemia major | Hemophagocytic lymphohistiocytosis (HLH) |
| Hemoglobinopathies | Rheumatoid arthritis (Felty’s syndrome) |
| Paroxysmal nocturnal hemoglobinuria | Systemic lupus erythematosus |
| Pernicious anemia | Collagen vascular diseases |
| Immune hyperplasia | Serum sickness |
| Response to infection (viral, bacterial, fungal, parasitic) | Immune hemolytic anemias |
| Infectious mononucleosis | Immune thrombocytopenias |
| AIDS | Immune neutropenias |
| Viral hepatitis | Drug reactions |
| Cytomegalovirus | Angioimmunoblastic lymphadenopathy |
| Subacute bacterial endocarditis | Sarcoidosis |
| Bacterial septicemia | Thyrotoxicosis (benign lymphoid hypertrophy) |
| Congenital syphilis | Interleukin 2 therapy |
| Splenic abscess | Extramedullary hematopoiesis |
| Tuberculosis | Myelofibrosis |
| Histoplasmosis | Marrow damage by toxins, radiation, strontium |
| Malaria | Marrow infiltration by tumors, leukemias, Gaucher’s disease |
| Enlargement Due to Abnormal Splenic or Portal Blood Flow | |
| Cirrhosis | Splenic artery aneurysm |
| Hepatic vein obstruction | Hepatic schistosomiasis |
| Portal vein obstruction, intrahepatic or extrahepatic | Congestive heart failure |
| Cavernous transformation of the portal vein | Hepatic echinococcosis |
| Splenic vein obstruction | Portal hypertension (any cause including the above): “Banti’s disease” |
| Infiltration of the Spleen | |
| Intracellular or extracellular depositions | Hodgkin’s disease |
| Amyloidosis | Myeloproliferative syndromes (e.g., polycythemia vera, essential thrombocytosis) |
| Gaucher’s disease | Angiosarcomas |
| Niemann-Pick disease | Metastatic tumors (melanoma is most common) |
| Tangier disease | Eosinophilic granuloma |
| Hurler’s syndrome and other mucopolysaccharidoses | Histiocytosis X |
| Hyperlipidemias | Hamartomas |
| Benign and malignant cellular infiltrations | Hemangiomas, fibromas, lymphangiomas |
| Leukemias (acute, chronic, lymphoid, myeloid, monocytic) | Splenic cysts |
| Lymphomas | |
| Unknown Etiology | |
| Idiopathic splenomegaly | Iron-deficiency anemia |
| Berylliosis | |
Blood disorders: Blood disorders such as leukemia, lymphoma, and sickle cell anemia
rheumatoid arthritis and systemic lupus erythematosus, as well as certain types of cancers that can spread to the spleen.
Type 1 Gauchers Disease
Reference
1. Longo DL. Enlargement of Lymph Nodes and Spleen. In: Loscalzo J, Fauci A, Kasper D, Hauser S, Longo D, Jameson J. eds. Harrison's Principles of Internal Medicine, 21e. McGraw-Hill Education; 2022. Accessed October 18, 2024. https://accessmedicine.mhmedical.com/content.aspx?bookid=3095§ionid=262792058