An acquired factor VIII inhibitor responsive to high-dose gamma globulin.
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 VII: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 VIII: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.
D001323:Autoantibodies / Q000032:analysis; D003161:Compartment Syndromes / Q000097:blood / Q000209:etiology* / Q000628:therapy; D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000276:immunology; D005260:Female; D006470:Hemorrhage / Q000097:blood / Q000209:etiology* / Q000628:therapy; D006801:Humans; D007116:Immunization, Passive; D008875:Middle Aged; D005719:gamma-Globulins / Q000008:administration & dosage*
D001323:Autoantibodies; D005719:gamma-Globulins; D005169:Factor VIII