Chapter 28: Hypersplenism and Hyposplenism

Congestive

1. Right-sided congestive heart failure

2. Budd-Chiari syndrome (inferior vena cava and hepatic vein thrombosis)

3. Cirrhosis with portal hypertension

4. Portal or splenic vein thrombosis

Immunologic

1. Viral infection

   1. Acute HIV infection/chronic infection

   2. Acute mononucleosis

   3. Dengue fever

   4. Rubella (rare except newborns)

   5. Cytomegalovirus infection (rare except newborns)

   6. Herpes simplex (rare except newborns)

2. Bacterial infection

   1. Subacute bacterial endocarditis

   2. Brucellosis

   3. Tularemia

   4. Melioidosis

   5. Listeriosis

   6. Plague

   7. Secondary syphilis

   8. Relapsing fever

   9. Psittacosis

   10. Ehrlichiosis

   11. Rickettsial diseases (scrub typhus, Rocky Mountain spotted fever, Q fever)

   12. Tuberculosis

   13. Splenic abscess (most common organisms are Enterobacteriaceae, Staphylococcus aureus, streptococcus group D, and anaerobic organisms as part of mixed flora infections)

3. Fungal Infection

   1. Blastomycosis

   2. Histoplasmosis

   3. Systemic candidiasis and hepatosplenic candidiasis

4. Parasitic infection

   1. Malaria

   2. Kala-azar

   3. Leishmaniasis

   4. Schistosomiasis

   5. Babesiosis

   6. Coccidioidomycosis

   7. Paracoccidioidomycosis

   8. Trypanosomiasis (cruzi, brucei)

   9. Toxoplasmosis (rare except newborns)

   10. Echinococcosis

   11. Cysticercosis

   12. Visceral larva migrans (Toxocara infection)

5. Inflammatory/autoimmune

   1. Systemic lupus erythematosus (SLE)

   2. Felty syndrome

   3. Juvenile rheumatoid arthritis

   4. Autoimmune lymphoproliferative syndrome (ALP syndrome)

   5. Hemophagocytic syndrome

   6. Common variable immunodeficiency

   7. Anti-D immunoglobulin administration

Associated with hemolysis

1. Thalassemia major and intermedia

2. Pyruvate kinase deficiency

3. Hereditary spherocytosis

4. Autoimmune hemolytic anemia (rare)

5. Sickle cell disease, more common in early childhood (splenic sequestration), hemoglobin C disease, and some other hemoglobinopathies

Infiltrative

1. Nonmalignant

   1. Splenic hematoma (splenic cysts are usually a late complication of a hematoma)

   2. Littoral cell angioma

   3. Disorders of sphingolipid metabolism

      1. Gaucher disease

      2. Niemann-Pick disease

   4. Cystinosis

   5. Amyloidosis (light-chain amyloid and amyloid A protein)

   6. Multicentric Castleman disease

   7. Mastocytosis

   8. Hypereosinophilic syndrome

   9. Sarcoidosis

2. Extramedullary hematopoiesis

   1. Primary myelofibrosis

   2. Osteopetrosis (childhood)

   3. Thalassemia major

3. Malignant

   1. Hematologic

      1. Chronic lymphocytic leukemia (especially prolymphocytic variant)

      2. Chronic myeloid leukemia

      3. Polycythemia vera

      4. Hairy cell leukemia

      5. Heavy chain disease

      6. Hepatosplenic lymphoma

      7. Acute leukemia (acute lymphoblastic leukemia/acute myeloid leukemia)

      8. Hodgkin and other lymphomas

   2. Nonhematologic

      1. Metastatic carcinoma (rare)

      2. Neuroblastoma

      3. Wilms tumor

      4. Leiomyosarcoma

      5. Fibrosarcoma

      6. Malignant fibrous histiocytoma

      7. Kaposi sarcoma

      8. Hemangiosarcoma

      9. Lymphangiosarcoma

      10. Hemangioendothelial sarcoma

Iatrogenic

1. Granulocyte colony-stimulating factor administration

2. Erythropoietin administration

Myeloproliferative disorders

1. Primary myelofibrosis

2. Chronic myeloid leukemia

Lymphomas

1. Hairy cell leukemia

2. Chronic lymphocytic leukemia (especially prolymphocytic variant)

Infectious