Chronic defibrination syndrome and thrombocytopenia in splenic hamartoma (splenoma)

In a 41-year-old woman who had suffered from hypofibrinogenemia, thrombocytopenia, leukopenia and splenomegaly for several years a causative hamartoma of the spleen (splenoma) was removed surgically. The results of a preoperative therapeutic trial with heparin, the marked accumulation of 125I-activity in the splenoma following intravenous injection of radioiodinated fibrinogen, the immunohistochemical demonstration of extensive fibrinogen deposits in the splenoma, and normalization of fibrinogen levels and blood cell counts after surgery point to the pathogenetic role of this rare splenic tumor. The salient clinical and pathological features of 41 splenoma cases in the literature are briefly reviewed. Hematologic signs were observed in only 9 splenoma patients.