Acquired Hemophilia Associated with Autoimmune Bullous Diseases: A Report of Two Cases and a Review of the Literature
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概要
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Acquired hemophilia (AHA) is a relatively rare and life-threatening disease caused by autoantibodies against factor VIII. Autoimmune bullous diseases (ABD) are also caused by autoantibodies against specific skin proteins. We herein report two cases of AHA associated with ABD. These coincidences are extremely rare, and only 14 documented cases have been reported previously. We further analyzed the properties of the autoantibodies in our patients. The epitopes were the A2 domain in patient 1, and both the A2 domain and the light chain in patient 2. Their isoforms were predominantly IgG4. Cross-reactivity could not be demonstrated. An accumulation of cases is required to unveil the pathogenesis of AHA.
著者
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Danbara Mikio
Department Of Hematology Kitasato University School Of Medicine
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Katayama Takuji
Department of Internal Medicine, Omiya Medical Center, Jichi Medical University
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Higashihara Masaaki
Department Of Hematology Internal Medicine Kitasato University School Of Medicine
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Watarai Akira
Department Of Dermatology Kitasato University School Of Medicine
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MIYAZAKI Koji
Department of Applied Chemistry, Tokyo Metropolitan University
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Aida Azusa
Department of Hematology, Kitasato University School of Medicine, Japan
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Makita Shinichi
Department of Hematology, Kitasato University School of Medicine, Japan
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Aoki Takumi
Department of Hematology, Kitasato University School of Medicine, Japan
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Katayama Takuji
Department of Hematology, Kitasato University School of Medicine, Japan
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Katayama Takuji
Department of Hematology, Internal Medicine, Kitasato University School of Medicine, Japan
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Miyazaki Koji
Department of Hematology, Kitasato University School of Medicine, Japan
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Higashihara Masaaki
Department of Hematology, Kitasato University School of Medicine, Japan
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