The spleen in the spleen syndrome: the association of splenoma with hematopoietic and neoplastic disease--compendium of cases since 1864.

Splenomas have been described as benign congenital growths. We report six cases of splenomas, all in males. Three had hairy cell leukemia, one had Hodgkin's disease, and one had myeloid metaplasia. Average splenic weight was 1629 gm, and all had multiple splenomas (average diameter 1.89 cm). Splenomas bulged above the cut splenic surface, were pseudoencapsulated, and showed involvement of the underlying disease. A re-evaluation of 105 prior cases are adult (86%; age: 49), with 56% associated with neoplastic disease or hematologic abnormalities. Average spleen weight was 626 gm, and 22% contained multiple splenomas. We conclude that splenomas are most likely an acquired proliferative process strongly associated with neoplastic disease or hematologic abnormalities. Splenomas can also be congenital growths (hamartoma; 26% of the series), which are of little importance.