Nontropical idiopathic splenomegaly

We have reported the case of a 47-year-old man who had massive splenomegaly and marked pancytopenia for which no cause was determined. Splenectomy produced dramatic improvement. The clinical, hematologic, and pathologic findings are characteristic of nontropical idiopathic splenomegaly, or Dacie's syndrome. In 20% of the 46 cases described in the literature, malignant lymphoma has subsequently developed.