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The Journal of Bone and Joint Surgery 78:1409-14 (1996)
© 1996 The Journal of Bone and Joint Surgery, Inc.

Operative Treatment of Intramedullary Hematoma Associated with Congenital Deficiency of {alpha}2-Plasmin Inhibitor. A Report of Three Cases*

YOSHIZUMI MIYAUCHI, M.D.{dagger}, YOSHIO MII, M.D.{dagger}, MAKOTO AOKI, M.D.{dagger}, SUSUMU TAMAI, M.D.{dagger}, YUKIHIRO TAKAHASHI, M.D.{dagger} and AKIRA YOSHIOKA, M.D.{dagger}, KASHIHARA CITY, NARA, JAPAN

Investigation performed at the Departments of Orthopedic Surgery and Pediatrics, Nara Medical University, Kashihara City, Nara


   Introduction
 
Congenital deficiency of {alpha}2-plasmin inhibitor is a very rare disease that is associated with severe bleeding problems. To our knowledge, the condition was first reported in 1978 by Koie et al., who described a twenty-five-year-old man who had symptoms similar to those of severe hemophilia. Since then, the cases of at least eight patients, from six families, who had homozygous deficiency have been reported3,7,8,10,12,17. The main symptoms were umbilical bleeding, bleeding in the central nervous system, epistaxis, bleeding gums, hemothorax, hypermenorrhea, hemarthrosis, and excessive loss of blood after minor trauma. These patients often needed to be hospitalized for long periods because of the tendency for severe bleeding that had begun in early childhood and was clinically similar to hemophilia. The episodes of bleeding were thought to be characterized by hemostatic plugs that had formed normally but disappeared rapidly because of unrestrained fibrinolysis. Thus, routine laboratory studies revealed no important . . . [Full Text of this Article]


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