Cystic lymphangioma of the spleen associated with hypersplenism

A rare case of cystic lymphangioma of the spleen accompanying hypersplenism syndrome is described. After stressing the importance of certain examinations such as arteriography and splenic scintigraphy in diagnosis the condition, the many ways in which cystic lymphangioma of the spleen may present clinically are reviewed; these go from asymptomatic to straightforward splenomegaly, with or without clinical signs, up to a clear-cut hypersplenism syndrome. The present case merits consideration for three reasons: 1) The absolutely exceptional nature of cystic lymphangioma of the spleen; 2) Exceptional association with hypersplenism syndrome; 3) Remission of the syndrome following splenectomy. It is concluded that, notwithstanding the condition's rarity, cystic lymphangioma of the spleen should be considered in the differential diagnosis of splenomegalic syndromes when these are also accompanied by hypersplenism.