Unidad de Hemostasia y Trombosis, Hospital Universitario y Politécnico La Fe, Valencia, Spain.
Haemophilia. 2013 Nov;19(6):920-5. doi: 10.1111/hae.12203. Epub 2013 Jun 4.
Measuring von Willebrand factor (VWF) activity is essential for the diagnosis of von Willebrand disease (VWD). The VWF activity is usually assessed based on measurement of the ristocetin cofactor (VWF:RCo). However, that test is technically challenging and has high intra- and inter-assay variabilities. A new automated chemiluminescent immunoassay VWF activity has recently become commercially available (HemosIL AcuStar von Willebrand Factor Ristocetin Cofactor Activity). The main objective of this study was to evaluate this new method and to compare it with the VWF:RCo assay as the reference method. We studied 91 samples, 18 healthy volunteers samples and 73 samples from patients (VWF:RCo level <50 IU dL(-1) ): 29 type 1 VWD, 13 type 2A, 5 type 2B, 5 type 2M, 3 type 2N, 5 type 3, 4 type 3 under treatment, 5 type 3 carriers and 4 samples with other pathologies. The HemosIL AcuStar VWF:RCo assay was 96% sensitive and 100% specific for detecting VWF abnormalities. The good analytical performance, and the sensitivity and specificity of HemosIL AcuStar VWF:RCo to detect VWF deficiency renders it a suitable method for VWD screening.
测定血管性血友病因子(VWF)活性对于血管性血友病(VWD)的诊断至关重要。VWF 活性通常通过测量瑞斯托霉素辅因子(VWF:RCo)来评估。然而,该检测技术具有挑战性,且具有较高的内、间检测变异性。一种新的自动化化学发光免疫分析法 VWF 活性最近已商业化(HemosIL AcuStar 血管性血友病因子瑞斯托霉素辅因子活性)。本研究的主要目的是评估该新方法,并将其与 VWF:RCo 测定法(参考方法)进行比较。我们研究了 91 个样本,包括 18 个健康志愿者样本和 73 个来自患者的样本(VWF:RCo 水平<50IU/dL(-1)):29 例 1 型 VWD、13 例 2A 型、5 例 2B 型、5 例 2M 型、3 例 2N 型、5 例 3 型、4 例正在接受治疗的 3 型、5 例 3 型携带者和 4 例其他病理样本。HemosIL AcuStar VWF:RCo 测定法对检测 VWF 异常的敏感性为 96%,特异性为 100%。HemosIL AcuStar VWF:RCo 具有良好的分析性能,对 VWF 缺乏的敏感性和特异性使其成为 VWD 筛查的合适方法。