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

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.

• Authors: Y Takahashi, T Tanaka, N Nakajima, A Yoshioka, H Fukui, Y Miyauchi, Y Mii, S Tamai
• Journal: Haemostasis (Haemostasis) Vol. 21 Issue 5 Pg. 321-7 ( 1991) ISSN: 0301-0147 [Print] Switzerland
• PMID: 1806461 (Publication Type: Case Reports, Journal Article)

Chemical References

• alpha-2-Antiplasmin
• Tranexamic Acid

Topics

• Adolescent
• Blood Loss, Surgical (prevention & control)
• Bone Diseases (diagnostic imaging, etiology, surgery)
• Child
• Female
• Femur
• Hematoma (diagnostic imaging, etiology, surgery)
• Humans
• Humerus
• Radiography
• Radius
• Tranexamic Acid (therapeutic use)
• alpha-2-Antiplasmin (deficiency)