Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.
Haemophilia. 2014 Jan;20(1):147-53. doi: 10.1111/hae.12264. Epub 2013 Sep 12.
von Willebrand disease (VWD) is caused by a quantitative and/or qualitative deficiency of the von Willebrand factor (VWF). The laboratory diagnosis of VWD is dependent on the measurement of VWF antigen (VWF:Ag) and ristocetin cofactor activity (VWF:RCo). The aim of this study was to undertake a two-centre evaluation of two new automated VWF:Ag and VWF:RCo assays systems from Instrumentation Laboratory (Bedford, USA). Using the two new analytical systems that operated with different detection principles: immunoturbidimetric (TOP500 analyser) and chemiluminescent (AcuStar analyser), VWF:Ag and VWF:RCo levels were determined in samples from 171 healthy normal subjects, 80 VWD patients (16 type 1, 58 type 2 and 6 type 3) and 7 acquired von Willebrand syndrome patients. With commercial lyophilized normal and pathological plasmas VWF: Ag and VWF:RCo assays performed on both analysers exhibited low levels of inter-assay imprecision (AcuStar: CV% range 3.3-6.9; TOP500: CV% range 2.6-6.3). Samples from normal healthy subjects (range: VWF:Ag 44.6-173.9 IU dL(-1) ; VWF:RCo 43.1-191.5 IU dL(-1)) and patients (range: VWF:Ag <0.3-115.1 IU dL(-1) ; VWF:RCo <0.5-57.2 IU dL(-1)) showed a good correlation between the two VWF:Ag and VWF:RCo methods (rs = 0.92 and 0.82 respectively), with only a few inconsistent cases among the patients' samples evaluated. The chemiluminescent assays had a lower limit of detection for both VWF:Ag and VWF:RCo compared to immunoturbidimetric tests (0.3 IU dL(-1) vs. 2.2 IU dL(-1) and 0.5 IU dL(-1) vs. 4.4 IU dL(-1) respectively). The TOP500 and AcuStar VWF:Ag and VWF:RCo assays were precise and compare well between centres, making these systems suitable for the diagnosis of VWD in non-specialized and reference laboratories.
血管性血友病（VWD）是由血管性血友病因子（VWF）的定量和/或定性缺乏引起的。VWD 的实验室诊断依赖于 VWF 抗原（VWF：Ag）和瑞斯托霉素辅因子活性（VWF：RCo）的测量。本研究的目的是在两个中心评估两个来自 Instrumentation Laboratory（美国贝德福德）的新型自动化 VWF：Ag 和 VWF：RCo 分析系统。使用两种新的分析系统，它们采用不同的检测原理：免疫比浊（TOP500 分析仪）和化学发光（AcuStar 分析仪），在 171 名健康正常受试者、80 名 VWD 患者（16 型 1、58 型 2 和 6 型 3）和 7 名获得性血管性血友病综合征患者的样本中测定了 VWF：Ag 和 VWF：RCo 水平。使用商业冻干正常和病理血浆，在两种分析仪上进行的 VWF：Ag 和 VWF：RCo 测定均表现出较低的批内精密度（AcuStar：CV%范围 3.3-6.9；TOP500：CV%范围 2.6-6.3）。来自正常健康受试者的样本（范围：VWF：Ag 44.6-173.9 IU dL(-1)；VWF：RCo 43.1-191.5 IU dL(-1)）和患者（范围：VWF：Ag <0.3-115.1 IU dL(-1)；VWF：RCo <0.5-57.2 IU dL(-1)）显示两种 VWF：Ag 和 VWF：RCo 方法之间具有良好的相关性（rs = 0.92 和 0.82），只有少数患者样本评估中存在不一致的情况。与免疫比浊试验相比，化学发光试验对 VWF：Ag 和 VWF：RCo 的检测下限更低（分别为 0.3 IU dL(-1) 对 2.2 IU dL(-1) 和 0.5 IU dL(-1) 对 4.4 IU dL(-1)）。TOP500 和 AcuStar VWF：Ag 和 VWF：RCo 测定具有良好的精度，且在中心之间比较一致，这使得这些系统适合在非专业和参考实验室中诊断 VWD。
