Enlarged spleen syndrome.

Enlarged spleen without a clear-cut etiology, and believed to be related to malarial infestations, has been referred to as "tropical splenomegaly" and "cryptogenetic splenomegaly." Splenectomy performed in such cases after a failure of antimalarial therapy shows histopathologically, while some of these meet the criteria for tropical splenomegaly, that most were cases of splenic abscess in various stages of formation, and some were lymphosarcoma. In sickle cell disease, one sequence of events involving the spleen is intermittent painful splenic infarction leading to splenic abscess or shrunken, fibrotic, and probably functionless spleen. It is proposed that an elective splenectomy may be indicated in cases of sickle cell disease to forestall this outcome. It is also proposed that an enlarged spleen that fails to respond to antimalarial medication after a certain period should be removed to establish the correct diagnosis, to prevent rupture, and to determine definitive management.