Geisen Ulrich, Zieger Barbara, Nakamura Lea, Weis Andreas, Heinz Jürgen, Michiels Jan Jacques, Heilmann Claudia
Institute of Clinical Chemistry and Laboratory Medicine, Freiburg, Germany.
Laboratory for Hemostaseology, Department of Pediatrics and Adolescent Medicine, Freiburg, Germany.
Thromb Res. 2014 Aug;134(2):246-50. doi: 10.1016/j.thromres.2014.04.033. Epub 2014 May 17.
Ristocetin cofactor activity of Von Willebrand factor (VWF:RCo) and the ratio VWF:RCo to its antigen VWF:Ag are used as routine screening to estimate VWF function and to detect types of Von Willebrand disease (VWD) caused by loss of high molecular weight multimers. However, the VWF:RCo test is prone to analytic imprecisions due to various reasons. We compared an assay for VWF activity (VWF:Ac) with VWF:RCo putting emphasis on the ratios to VWF:Ag.
We evaluated 942 samples from 432 patients and evaluated three groups in detail: normal patients (normal multimers, VWF:Ag and VWF:RCo >0.5 U/ml, VWD type 1 excluded; n=258), VWD type 1 (n=76) and acquired Von Willebrand syndrome (AVWS, n=326). In addition, 30 healthy subjects were analysed.
VWF:Ac and VWF:RCo correlated well (Pearson´s r=0.96, p<0.01), so did their ratios to VWF:Ag (Pearson´s r=0.82, p<0.01). We calculated the normal range of VWF:Ac/VWF:Ag for healthy subjects as 0.8-1.16. In comparison, the reference range (mean±2std) derived from normal patient samples was 0.73-1.14. The corresponding ranges for VWF:RCo/VWF:Ag came to 0.74-1.23 (healthy) and 0.62-1.25 (normal patients). The ratios showed similar results regarding VWD type 1. The sensitivity for AVWS was higher with VWF:Ac/VWF:Ag than with VWF:RCo/VWF:Ag (97.5% versus 84.7%).
The data suggest that the results obtained with the VWF:Ac assay are comparable to that of the VWF:RCo assay. An AVWS was more reliably detected by VWF:Ac/VWF:Ag. We assume that the VWF:Ac assay could replace VWF:RCo for routine screening for AVWS, especially when prompt evaluation is required.
血管性血友病因子的瑞斯托霉素辅因子活性(VWF:RCo)以及VWF:RCo与其抗原VWF:Ag的比值,被用作常规筛查手段,以评估VWF功能,并检测由高分子量多聚体缺失引起的血管性血友病(VWD)类型。然而,由于各种原因,VWF:RCo检测容易出现分析误差。我们比较了一种VWF活性检测方法(VWF:Ac)与VWF:RCo,并重点关注它们与VWF:Ag的比值。
我们评估了来自432例患者的942份样本,并详细评估了三组:正常患者(多聚体正常,VWF:Ag和VWF:RCo>0.5 U/ml,排除1型VWD;n = 258)、1型VWD(n = 76)和获得性血管性血友病综合征(AVWS,n = 326)。此外,还分析了30名健康受试者。
VWF:Ac与VWF:RCo相关性良好(Pearson相关系数r = 0.96,p<0.01),它们与VWF:Ag的比值也是如此(Pearson相关系数r = 0.82,p<0.01)。我们计算出健康受试者VWF:Ac/VWF:Ag的正常范围为0.8 - 1.16。相比之下,从正常患者样本得出的参考范围(均值±2标准差)为0.73 - 1.14。VWF:RCo/VWF:Ag的相应范围为0.74 - 1.23(健康受试者)和0.62 - 1.25(正常患者)。这些比值在1型VWD方面显示出相似的结果。VWF:Ac/VWF:Ag对AVWS的敏感性高于VWF:RCo/VWF:Ag(97.5%对84.7%)。
数据表明,VWF:Ac检测获得的结果与VWF:RCo检测结果相当。VWF:Ac/VWF:Ag能更可靠地检测出AVWS。我们认为,VWF:Ac检测可替代VWF:RCo用于AVWS的常规筛查,尤其是在需要快速评估时。