Hypersplenism is a cytopenia resulting from blood pooling in the spleen and is almost always associated with splenomegaly.
Pathology
Etiology
There is an almost overwhelming list, some more common causes are given below 1,3,4:
- congestive splenomegaly: cirrhosis, Budd-Chiari syndrome, portal vein obstruction
- infections: viral, malaria, tuberculosis
- inflammation: sarcoidosis, SLE
- chronic hemolytic disorders: hemolytic anemia, polycythemia rubra vera
- malignancy:
- hematological: myeloproliferative disorders, lymphoma, leukemia, Langerhans cell histiocytosis
- non-hematological: metastases, angiosarcoma, hemangioma
- storage disorders: Gaucher disease
- other: primary splenic hyperplasia, hyperthyroidism, wandering spleen
Treatment and prognosis
Hypersplenism does not often require treatment because the cytopenia is often not severe enough to cause significant bleeding 1. Splenectomy or splenic embolization can be considered if warranted.
Complications
- splenic rupture
- splenic volvulus
- hemorrhagic and infectious complications due to the pancytopenia
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