Blood Res.  2013 Mar;48(1):58-62. 10.5045/br.2013.48.1.58.

Characterization of anti-factor VIII antibody in a patient with acquired hemophilia A

Affiliations
  • 1Department of Internal Medicine, CHA University School of Medicine, Seongnam, Korea. doh@cha.ac.kr
  • 2Department of Laboratory Medicine, CHA University School of Medicine, Seongnam, Korea.
  • 3Department of Pediatrics, Nara Medical University School of Medicine, Nara, Japan. mshima@naramed-u.ac.jp

Abstract

Acquired hemophilia A (AHA) is a bleeding disorder caused by the development of an auto-antibody 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.

Keyword

Acquired hemophilia A; Factor VIII autoantibody; Epitope

MeSH Terms

Antibodies
Enzyme-Linked Immunosorbent Assay
Factor VIII
Female
Hemophilia A
Hemorrhage
Humans
Immunoblotting
Immunoglobulins
Korea
Light
Partial Thromboplastin Time
Phospholipids
von Willebrand Factor
Antibodies
Factor VIII
Immunoglobulins
Phospholipids
von Willebrand Factor
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