ITPに合併した抗VWF抗体によるvon Willebrand症候群  [in Japanese] Acquired von Willebrand syndrome in a patient with immune thrombocytopenic purpura  [in Japanese]

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Author(s)

Abstract

後天性von Willebrand syndrome(以下AVWS)は,先天性VWD類似の稀な疾患である。我々は,血小板数3.2万/μ<i>l</i>で著明な出血傾向を示した特発性血小板減少性紫斑症(ITP)に合併したAVWSの1例を報告する。症例は76歳女性。脳梗塞後遺症経過中ITPを発症1年後,全身の著明な出血傾向を示した。第VIII因子活性22%, VWF: RCo<6%, VWF: Ag276%, 第XIII因子活性42%, VWF large multimer (±)。ELISA法でIgG1とIgG4の抗VWF抗体を検出,ITPに合併したAVWSと診断し,プレドニゾロン20 mg/日開始後VWFは正常化,血小板数は増加しなかったが出血傾向は消失した。AVWSはリンパ増殖性疾患,骨髄増殖性疾患,心血管疾患に多いといわれ,ITPでの報告はない。本症例は,原因不明の第XIII因子欠乏症も合併した稀な例である。

Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder similar to inherited von Willebrand disease. We describe a 78-year-old woman with coexistent idiopathic thrombocytopenic purpura (ITP) and AVWS. The patient had once been admitted to our hospital because of cerebral infarction. Her platelet count had been normal at that time. Ten years later, she showed a severe bleeding tendency (platelet count 3.2×10<sup>4</sup>/μ<i>l</i>). Analysis of hemostatic parameters showed very low (<6%) von Willebrand factor ristocetin cofactor (vWF: Rco), and low VIII: C (22%), but elevated (276%) von Willebrand antigen. Electrophoretic analysis of plasma showed low levels of the high-molecular weight VWF multimer. The presence of antibodies (IgG1 and IgG4) to VWF was detected by enzyme linked immunosorbent assay (ELISA). Factor XIII activity was 42%. Treatment with corticosteroids did not improve the thrombocytopenia, but did correct the bleeding diathesis. Also, VWF: Rco and VIII: C showed normalization. These findings indicated that the patient had ITP associated with AVWS. All reported cases of AVWS associated with systemic lupus erythematosus were cured by appropriate treatment of the underlying autoimmune disease with prednisone or immunosuppression. This bleeding disorder occurs mainly in patients with lymphoproliferative, myeloproliferative, cardiovascular and immunologic disorders, but no patients with ITP have previously been reported. This patient had the rare presentation of AVWS complicated by ITP and factor XIII deficiency.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 56(7), 901-904, 2015

    The Japanese Society of Hematology

Codes

  • NII Article ID (NAID)
    130005093126
  • NII NACSIS-CAT ID (NCID)
    AN00252940
  • Text Lang
    JPN
  • ISSN
    0485-1439
  • NDL Article ID
    026636622
  • NDL Call No.
    Z19-295
  • Data Source
    NDL  J-STAGE 
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