Autoimmune coagulation factor V/5 deficiency during chronic disseminated intravascular coagulation

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  • ISHIMORI Noriko
    Department of Internal Medicine, Tsudanuma Central General Hospital
  • WAKABAYASHI Mutsumi
    Department of Internal Medicine, Tsudanuma Central General Hospital
  • SAKURAI Kenji
    Department of Internal Medicine, Tsudanuma Central General Hospital
  • SUDA Akira
    Department of Internal Medicine, Tsudanuma Central General Hospital
  • SOURI Masayoshi
    Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine Department of Public Health and Hygiene, Yamagata University School of Medicine
  • OSAKI Tsukasa
    Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine Department of Public Health and Hygiene, Yamagata University School of Medicine
  • ICHINOSE Akitada
    Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine

Bibliographic Information

Other Title
  • 慢性播種性血管内凝固症候群の経過中に発症した自己免疫性凝固第V/5因子欠乏症
  • 慢性播種性血管内凝固症候群の経過中に発症した自己免疫性凝固第V/5因子欠乏症 : 第16回日本血液学会関東甲信越地方会 優秀演題
  • マンセイ ハシュセイ ケッカン ナイ ギョウコ ショウコウグン ノ ケイカ チュウ ニ ハッショウ シタ ジコ メンエキセイ ギョウコ ダイV/5 インシ ケツボウショウ : ダイ16カイ ニホン ケツエキ ガッカイ カントウ コウシンエツチホウカイ ユウシュウ エンダイ

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Abstract

<p>Aortic regurgitation, a thoracoabdominal aortic aneurysm, chronic myeloid leukemia, and chronic kidney disease were all being treated at two hospitals for an 83-year-old man. He was admitted to the Department of Orthopedics at our hospital with a lumbar compression fracture. Later, he experienced melena, for which the Department of Internal Medicine was consulted. Due to the aberrant results of PT-INR (7.1) and a PTT > 200 seconds on a coagulation test, we suspected the presence of an autoimmune coagulation factor deficiency, and prednisolone immunosuppressive therapy medication was started right away. Due to a sharp decline in FV/5 activity, the presence of FV/5 inhibitors, and the presence of anti-FV/5 autoantibodies, a final diagnosis of autoimmune coagulation factor V (FV/5) deficiency was made. After the start of immunosuppressive therapy, the FV/5 inhibitor and anti-FV/5 autoantibodies disappeared, and the FV/5 activity progressively returned to normal. Disseminated intravascular coagulation—which may have been caused by a known aortic aneurysm—worsened while tapering off prednisolone. Due to the patient’s advanced age and other problems, the aneurysm was extensive and inappropriate for surgical repair. The coagulation test findings improved gradually upon initiation of warfarin therapy. Herein, the patient had autoimmune FV/5 deficiency, a rare disorder that made diagnosis and therapy difficult because of the patient’s several coexisting conditions.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 64 (2), 113-118, 2023

    The Japanese Society of Hematology

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