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KMID : 0366220130480010058
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Korean Journal of Hematology
2013 Volume.48 No. 1 p.58 ~ p.62
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Characterization of anti-factor VIII antibody in a patient with acquired hemophilia A
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Oh Ji-Su
Lim Yeong-Min
Jang Moon-Ju
Huh Ji-Young
Shima Midori
Oh Do-Yeun
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Abstract
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Acquired hemophilia A (AHA) is a bleeding disorder caused by the development of an autoantibody against endogenous factor VIII (FVIII). In this study, the epitope of the autoantibody was identified in a 67-year-old female patient with AHA. A prolonged activated partial thromboplastin time (77.4 s) that failed to correct in an incubation mixing test (68.2 s), a decreased FVIII activity, and a high FVIII inhibitor (14.6 Bethesda units/mL) were observed. Enzyme-linked immunosorbent assay demonstrated that the antibody belonged to the immunoglobulin G4 subclass. An immunoblotting assay revealed the light chain (A3/C1/C2 domain) of FVIII as the binding region of the antibody. The bleeding experienced by our patient resulted from the interference of FVIII binding to both FIX by anti-A3 antibodies and phospholipids and von Willebrand factor by anti-C2 antibodies. To the best of our knowledge, this is the first study in Korea characterizing an autoantibody in the context of AHA.
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KEYWORD
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Acquired hemophilia A, Factor VIII autoantibody, Epitope
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