An Acquired Factor VIII Inhibitor Responsive to High-Dose Gamma Globulin

Summary

A 55-year-old previously well woman noted easy bruising and developed a swollen, painful leg after minimal trauma. A compartment syndrome was diagnosed, and medial and lateral fasciotomies were performed with evacuation of a massive hematoma. However, blood rapidly reaccumulated in the wound. The VILI : C level was 2%, and 4 Bethesda units of factor VIII inhibitor were detected. After initial treatment with clotting factor concentrates and corticosteroids failed to control bleeding or reduce inhibitor titers, gamma globulin, 25 g daily for 5 days, was administered. The inhibitor became undetectable, VIII :C levels rose, and bleeding stopped. However, 5 days later VIII :C levels were again low and bleeding recurred. A second course of gamma globulin, 50 g daily for 2 days, was accompanied by a prompt increase in VIII :C, and uneventful recovery. In conclusion, in this patient with an autoantibody to Vili : C, a response to gamma globulin was observed on two occasions, and the second response came when steroids were being tapered and the patient was on no other medication.

Keywords

Factor VIII inhibitor - Gamma globulin - Circulating anticoagulant - Factor VIII autoantibody

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