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- Hypersplenism refers to overactivity of the spleen and presents as:
- Splenomegaly (commonly but not always)
- Cytopenias with respective bone marrow hyperplasia of precursors
- Resolution of the cytopenias with splenectomy (1)
- Hypersplenism can usually be diagnosed without splenectomy.
- Splenomegaly is not synonymous with hypersplenism. Overactivity of the spleen can occur without enlargement, as is seen in immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia.
May be as common as 30–70% in patients with cirrhosis and portal hypertension
Enlargement of the spleen results in sequestration of formed blood elements, leading to peripheral cytopenias and concomitant bone marrow precursor hyperplasia.
Many of the common etiologies are listed below. Almost any process involving the spleen or the hematologic system can result in hypersplenism:
- Myeloproliferative disorders
- Polycythemia vera
- Primary hypersplenism
- Autoimmune hemolytic anemia
- Hematologic malignancies
- Various carcinomas
- Storage diseases:
- Gaucher disease
- Niemann-Pick disease
- Glycogen storage disease
- Systemic lupus erythematosus
- Felty syndrome