Development of acquired hemophilia A during maintenance therapy for immune thrombocytopenia

  • OGAWA Yoshiyuki
    Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine
  • YANAGISAWA Kunio
    Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine
  • ISHIZAKI Takuma
    Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine
  • NAITO Chiaki
    Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine
  • MIHARA Masahiro
    Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine
  • HANDA Hiroshi
    Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine
  • SHIZUKA Reiko
    Clinical Laboratory Center, Gunma University Hospital
  • INOUE Madoka
    Clinical Laboratory Center, Gunma University Hospital
  • NAITO Sumiyoshi
    Department of Clinical Laboratory, School of Dentistry, Health Sciences University of Hokkaido
  • IEKO Masahiro
    Department of Internal Medicine, School of Dentistry, Health Sciences University of Hokkaido
  • ICHINOSE Akitada
    Department of Molecular Patho-Biochemistry, Yamagata University
  • NOJIMA Yoshihisa
    Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine

Bibliographic Information

Other Title
  • 免疫性血小板減少症の維持療法中に発症した後天性血友病A
  • 症例報告 免疫性血小板減少症の維持療法中に発症した後天性血友病A
  • ショウレイ ホウコク メンエキセイ ケッショウバン ゲンショウショウ ノ イジ リョウホウ チュウ ニ ハッショウ シタ コウテンセイ ケツユウビョウ A

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Abstract

Acquired hemophilia A (AHA) is a rare coagulation disorder caused by autoantibodies against coagulation factor VIII (FVIII). We report herein a very rare case of AHA complicated by immune thrombocytopenia (ITP). A 30-year-old woman was hospitalized with severe thrombocytopenia. Her platelet count was 5,000/μl on admission, at which time APTT was normal. ITP was diagnosed and she was treated with γ-globulin, platelet transfusion, and prednisolone at 1 mg/kg/day. She was discharged after platelet count normalization and prednisolone was tapered to 5 mg/day. During the prednisolone tapering, purpura appeared on both thighs and in the left inguinal region, and APTT was found to be prolonged. She was referred to our hospital for examination of APTT prolongation. FVIII activity was markedly decreased to 7.7% and the FVIII inhibitor was positive (1.5 BU/ml), based on which AHA was diagnosed. We carefully followed this patient without intensification of immunosuppressive therapy for 7 weeks, but her platelet count decreased from 150,000/μl to 70,000/μl and the FVIII inhibitor increased to 4 BU/ml. We therefore increased prednisolone to 30 mg/day, after which her platelet count increased and complete remission of AHA was achieved by day 42. In addition, we examined the relationship of the FVIII inhibitor and FVIII binding antibody in this case.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 57 (4), 456-460, 2016

    The Japanese Society of Hematology

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