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Haemostasis. 1991;21(5):321-7.

Intramedullary multiple hematomas in siblings with congenital alpha-2-plasmin inhibitor deficiency: orthopedic surgery with protection by tranexamic acid.

Author information

1
Department of Pediatrics, Nara Medical College, Kashihara, Japan.

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.

PMID:
1806461
DOI:
10.1159/000216243
[Indexed for MEDLINE]

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