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首页> 外文期刊>Journal of Thrombosis and Thrombolysis >Correlation between von Willebrand factor antigen, von Willebrand factor ristocetin cofactor activity and factor VIII activity in plasma
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Correlation between von Willebrand factor antigen, von Willebrand factor ristocetin cofactor activity and factor VIII activity in plasma

机译：血浆中血管性假血友病因子抗原，血管性假血友病因子瑞斯托菌素辅助因子活性与凝血因子VIII活性之间的相关性

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摘要

Background The laboratory diagnosis of von Willebrand Factor (VWF) deficiencies includes qualitative and quantitative measurements of VWF and clotting factor VIII (FVIII). Since the FVIII activity is frequently normal in patients with mild type 1 or 2 von Willebrand disease (VWD), there is controversy whether FVIII testing should accompany VWF Antigen (VWF:Ag) assay. Methods The aim of this study was to explore the correlation between VWF:Ag, VWF ristocetin cofactor activity (VWF:RCo) and FVIII in 213 consecutive patients undergoing screening for VWD. Results Forty-six patients were identified with VWF:Ag levels lower than the diagnostic threshold (54 IU/dl). A significant correlation was observed between VWF:Ag and VWF:RCo (r = 0.892; p < 0.001), VWF:Ag and FVIII (r = 0.834; p < 0.001), VWF:RCo and FVIII (r = 0.758; p < 0.001). Receiver operating characteristic curve analysis of the VWF:Ag assay revealed an area under the curve of 0.978 and 0.957 for detecting life-threatening values of FVIII (<30 IU/dl) and VWF:RCo (<40 IU/dl), respectively. The negative and positive predictive values at the VWF:Ag threshold value of 54 IU/dl were 100% and 33% for detecting life-threatening FVIII deficiencies, 94% and 80% for identifying abnormal values of VWF:RCo. Conclusions Due to the excellent correlation between VWF:Ag and FVIII and to the diagnostic efficiency of VWF:Ag for identifying abnormal FVIII levels in patients with VWF deficiency, routine measurement of FVIII may not be necessary in the initial screening of patients with suspected VWD. However, the limited negative predictive value of VWF:Ag for identifying type 2 VWD does not allow to eliminate VWF:RCo or VWF:FVIIIB assays from the diagnostic workout.
机译：背景von Willebrand因子（VWF）缺陷的实验室诊断包括VWF和凝血因子VIII（FVIII）的定性和定量测量。由于FVIII活性在轻度1型或2型von Willebrand病（VWD）患者中通常是正常的，因此是否应在VWF抗原（VWF：Ag）分析中同时进行FVIII检测存在争议。方法本研究旨在探讨连续213例接受VWD筛查的患者中VWF：Ag，VWF瑞斯托菌素辅助因子活性（VWF：RCo）和FVIII的相关性。结果鉴定出46例VWF：Ag水平低于诊断阈值（54 IU / dl）。在VWF：Ag和VWF：RCo（r = 0.892; p <0.001），VWF：Ag和FVIII（r = 0.834; p <0.001），VWF：RCo和FVIII（r = 0.758; p < 0.001）。 VWF：Ag测定的受试者工作特征曲线分析显示，曲线下的面积为0.978和0.957，分别用于检测威胁生命的FVIII（<30 IU / dl）和VWF：RCo（<40 IU / dl）的生命值。在VWF：Ag阈值为54 IU / dl时，阴性和阳性预测值分别为100％和33％（用于检测威胁生命的FVIII缺陷），94％和80％（用于识别VWF：RCo的异常值）。结论由于VWF：Ag与FVIII之间的良好相关性以及VWF：Ag对VWF缺乏症患者FVIII异常水平诊断的诊断效率，在初筛疑似VWD的患者中可能无需常规检测FVIII。但是，用于识别2型VWD的VWF：Ag阴性预测值有限，因此无法从诊断工作中消除VWF：RCo或VWF：FVIIIB分析。

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  • 来源
    《Journal of Thrombosis and Thrombolysis》 |2008年第2期|150-153|共4页
  • 作者

    Giuseppe Lippi; Massimo Franchini; Gian Luca Salvagno; Martina Montagnana; Giovanni Poli; Gian Cesare Guidi;

  • 作者单位

    Sezione di Chimica Clinica Dipartimento di Scienze Morfologico-Biomediche Università di Verona Ospedale Policlinico G.B. Rossi Piazzale Scuro 10 Verona 37134 Italy;

    Servizio di Immunoematologia e Trasfusione Azienda Ospedaliera di Verona Italy;

    Sezione di Chimica Clinica Dipartimento di Scienze Morfologico-Biomediche Università di Verona Ospedale Policlinico G.B. Rossi Piazzale Scuro 10 Verona 37134 Italy;

    Sezione di Chimica Clinica Dipartimento di Scienze Morfologico-Biomediche Università di Verona Ospedale Policlinico G.B. Rossi Piazzale Scuro 10 Verona 37134 Italy;

    Sezione di Chimica Clinica Dipartimento di Scienze Morfologico-Biomediche Università di Verona Ospedale Policlinico G.B. Rossi Piazzale Scuro 10 Verona 37134 Italy;

    Sezione di Chimica Clinica Dipartimento di Scienze Morfologico-Biomediche Università di Verona Ospedale Policlinico G.B. Rossi Piazzale Scuro 10 Verona 37134 Italy;

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  • 正文语种 eng
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  • 关键词

    von Willebrand factor; Factor VIII; Diagnosis; Screening;

    机译：von Willebrand因子;VIII因子;诊断;筛查;

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