Recurrence of acquired factor V inhibitor after four years of remission

  • AKASHI Naoki
    Department of Hematology, Gunma University Graduate School of Medicine
  • OGAWA Yoshiyuki
    Department of Hematology, Gunma University Graduate School of Medicine The Japanese Collaborative Research Group on Acquired Coagulopathies Supported by the Japanese Ministry of Health, Labor, and Welfare
  • YANAGISAWA Kunio
    Department of Hematology, Gunma University Graduate School of Medicine
  • OSAKI Yohei
    Department of Hematology, Gunma University Graduate School of Medicine
  • SHIMIZU Hiroaki
    Department of Hematology, Gunma University Graduate School of Medicine
  • ISHIZAKI Takuma
    Department of Hematology, Gunma University Graduate School of Medicine
  • INOUE Madoka
    Clinical Laboratory Center, Gunma University Hospital
  • MURAKAMI Masami
    Department of Clinical Laboratory Medicine, Gunma University Graduate School of Medicine
  • SOURI Masayoshi
    The Japanese Collaborative Research Group on Acquired Coagulopathies Supported by the Japanese Ministry of Health, Labor, and Welfare Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine
  • ICHINOSE Akitada
    The Japanese Collaborative Research Group on Acquired Coagulopathies Supported by the Japanese Ministry of Health, Labor, and Welfare Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine
  • HANDA Hiroshi
    Department of Hematology, Gunma University Graduate School of Medicine

Bibliographic Information

Other Title
  • 初回寛解から4年後に再燃した後天性凝固第V因子インヒビター
  • 症例報告 初回寛解から4年後に再燃した後天性凝固第Ⅴ因子インヒビター
  • ショウレイ ホウコク ショカイカンカイ カラ 4ネンゴ ニ サイネン シタ コウテンセイ ギョウコ ダイ Ⅴ インシ インヒビター

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Abstract

<p>Acquired factor V inhibitor (AFV-I) is a rare bleeding disorder wherein autoantibodies are developed against coagulation factor V (FV). The clinical symptoms are variable, from laboratory abnormalities without bleeding to life-threatening hemorrhage. We report herein the case of a patient with AFV-I with two relapses 4 years after the first remission. A 66-year-old male was diagnosed with AFV-I in March 20XX−4. He was treated with prednisolone (PSL) at 50 mg/day and achieved remission within 1 month. PSL dose was tapered to oral administration of 2.5 mg every other day, and long-term remission was maintained. He had been treated with dual antiplatelet therapy (DAPT) for old myocardial infarction. FV activity was markedly reduced to 3.4%, and FV inhibitor was detected (1.0 BU/ml) in May 20XX. We followed the patient without increasing the treatment dose for 2 months, but no spontaneous improvement was seen. Because DAPT was ongoing, we judged that the bleeding risk was high, although only minor bleeding symptoms appeared. PSL was therefore increased to 40 mg/day in June. FV inhibitor rapidly disappeared. When PSL dose was gradually decreased, FV activity decreased, and subcutaneous bleeding occurred in February 20XX+1. PSL dose was increased again for the second relapse, and the patient achieved remission. Few reports have described recurrent AFV-I, and no cases of two relapses have been reported. We believe that this case report is useful for examining the long-term management of AFV-I.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 60 (1), 46-50, 2019

    The Japanese Society of Hematology

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