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Clinical significance of measurement for vWF cleaving protease activity

Research Project

Project/Area Number 13672428
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Laboratory medicine
Research InstitutionKeio University

Principal Investigator

KAWAI Yohko  M.D. Keio University, School of Medicine, Assistant Professor, 医学部, 講師 (00129727)

Project Period (FY) 2001 – 2002
Project Status Completed (Fiscal Year 2002)
Budget Amount *help
¥3,000,000 (Direct Cost: ¥3,000,000)
Fiscal Year 2002: ¥1,200,000 (Direct Cost: ¥1,200,000)
Fiscal Year 2001: ¥1,800,000 (Direct Cost: ¥1,800,000)
Keywordsvon Willebrand factor(vWF) / vWF-Cleaving protease(vWF-CP) / allo-BMT / endothelial perturbation / Thrombotic microangiopathy(TMA) / metalloprotease / ADAMTS 13 / Veno-occulusive disease (VOD) / ADAMTS 13 / VWF / VWF切断酵素 / 血小板減少 / 破砕赤血球 / 溶血性貧血 / 自己抗体 / 微小血管障害
Research Abstract

Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia associated with red cell fragmentation, and neurological and renal symptoms. Plasma from patients with TTP has been shown to contain unusually large von Willebrand factor (vWF) multimers that may cause platelet agglutination in vivo. Recently, a metalloprotease responsible for the cleavage of vWF multimers has been isolated from normal human plasma and was found to be deficient in some patients with TTP. We examined the activity of vWF-cleaving protease (vWF-CP), by modified Furlan's method, in plasma from 1 patient with familial TTP, 3 with acquired TTP, 4 with thrombotic microangiopathy(TMA) and 2 with veno-occlusive disease (VOD) associated after allo-BMT. Diluted plasma samples of patients were incubated with protease-free vWF purified from normal human plasma, in the presence of urea and barium ions. The extent of vWF degradation was assayed by e … More lectrophoresis using SDS-agarose gels and immunoblot techniques. Activity of vWF-CP from 12 normal plasma samples has been shown to be 77 - 180% (average 115%), whereas, no vWF-CP(below 5%) was observed in plasma from the single case with familial TTP, before and after plasma exchange, although FFP infusion therapy was effective for this patient in recovering from thrombocytopenia. In the 3 cases of acquired TTP, 2 patients showed lack of vWF-CP activity in plasma, and inhibitors against vWF-CP have been elucidated by plasma cross-mixing test. After extensive plasma exchange and FFP infusion followed by corticosteroid therapy, normal vWF-CP was recovered in plasma from the 2 acquired TTP patients. Among BMT patients, plasma from the 4 BMT-TMA cases showed normal vWF-CP activities of 55 - 111%, whereas plasma from the 2 cases of BMT-VOD revealed low vWF-CP activity, with 24% and 37%, respectively. Thus, measurement of vWF-CP is crucial to predict differentiation of primary forms of TMA to establish the pathogenesis in varied endothelial dysfunction Less

Report

(3 results)
  • 2002 Annual Research Report   Final Research Report Summary
  • 2001 Annual Research Report
  • Research Products

    (21 results)

All Other

All Publications (21 results)

  • [Publications] 片桐尚子, 川合陽子 他: "血栓症微小血管症におけるvon Willebrand因子切断酵素活性測定の臨床的意義"臨床病理. 51(8)(in press). (2003)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] 川合陽子: "血管における血栓制御機構と内皮細胞障害マーカー"日本臨床検査自動化学会会誌. 27(5). 619-627 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] 川合陽子: "血管内皮細胞由来の血栓関連因子とずり応力"血栓と循環. 9(4). 412-413 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] 川合陽子: "血液検査の標準化"臨床検査. 46(8). 887-891 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] 川合陽子: "特発性血小板減少性紫斑病"medicina. 38(13). 2034-2036 (2001)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] 川合陽子, 渡辺清明: "血管内皮細胞障害の分子マーカー"Medical Practice. 17(2). 270-271 (2000)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Katagiri,H, Kawai,Y et al.: "Measurement of plasma von Willebrand factor cleaving protease in patients with varied thrombotic microangiopathy"J Lab Med. 51(in press). (2003)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Kawai,Y: "Vascular control of hemostasis under flow, and molecular markers for perturbed endothelium."JJCLA. 27. 619-627 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Kawai,Y: "Effect of shear stress on endothelium-derived hemostatic factors"Thrombosis & Circulation. 9. 412-413 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Kawai,Y: "Standardization of hematological laboratory tests"J Med Tech. 46. 887-891 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Kawai,Y: "Idiopathic thrombocytopenic purpura"Medicina. 38. 2034-2036 (2001)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Kawai,Y, Watanabe,K: "Molecular markers for perturbed human endothelium"Medical Practice. 17. 270-271 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] 片桐尚子, 川合陽子, 他: "血栓性微小血管症におけるvon Willebrand因子切断酵素活性測定の臨床的意義"臨床病理. 51(8)(in press). (2003)

    • Related Report
      2002 Annual Research Report
  • [Publications] 川合陽子: "血管における血栓制御機構と内皮細胞障害マーカー"日本臨床検査自動化学会会誌. 27(5). 619-627 (2002)

    • Related Report
      2002 Annual Research Report
  • [Publications] 川合陽子: "血管内皮細胞由来の血栓関連因子とずり応力"血栓と循環. 9(4). 412-413 (2002)

    • Related Report
      2002 Annual Research Report
  • [Publications] 川合陽子: "血液検査の標準化"臨床検査. 46(8). 887-891 (2002)

    • Related Report
      2002 Annual Research Report
  • [Publications] 川合陽子: "特発性血小板減少性紫斑病"medicina. 38(13). 2034-2036 (2001)

    • Related Report
      2002 Annual Research Report
  • [Publications] 川合陽子, 渡辺清明: "血管内皮細胞障害の分子マーカー"Medical Practice. 17(2). 270-271 (2000)

    • Related Report
      2001 Annual Research Report
  • [Publications] 川合陽子: "表面マーカー検査"Medical Technology. 29. 279-287 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] 川合陽子, 清水長子: "見逃されやすい血小板形態の観察"日本検査血液学会雑誌. 2. 76-84 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] 川合陽子, 清水長子: "診断に役立つ赤血球形態の観察"日本検査血液学会雑誌. 1. 223-231 (2000)

    • Related Report
      2001 Annual Research Report

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Published: 2001-04-01   Modified: 2016-04-21  

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