Abstract
Congenital alpha 2-plasmin inhibitor deficiency is very rare, but causes bleeding problems similar to hemophilia. Three young Japanese sisters affected with congenital alpha 2-plasmin inhibitor deficiency have been reported by us earlier. Recently we encountered a particular form of intramedullary multiple hematomas in the long bones in all of them which was not reported previously. In 2 of the sisters orthopedic operations were successfully performed by using an antiplasmin reagent: tranexamic acid. In this paper we describe the characteristics of the hematomas and the hemostatic management with tranexamic acid.
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