Mohammed Soma, Favaloro Emmanuel J
Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, Westmead, 2145 NSW, Australia.
NSW Health Pathology, Westmead, 2145 NSW, Australia.
Methods Mol Biol. 2017;1646:435-451. doi: 10.1007/978-1-4939-7196-1_32.
von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders develop due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for these VWF-related disorders requires assessment of both VWF level and VWF activity, the latter requiring multiple assays because of the many functions carried out by VWF to help prevent bleeding. The current paper describes several protocols for assessment of VWF activity by means of VWF ristocetin cofactor (VWF:RCo). These assays identify VWF activity by quantitative assessment of VWF protein adhesion to platelets or other particles and subsequent detection of the adhered VWF as facilitated by inclusion of ristocetin. The most commonly performed assays for VWF:RCo comprise platelet agglutination assays, latex agglutination assays, and chemiluminescent assay (CLIA), with three of these described in this chapter.
据报道，血管性血友病（VWD）是最常见的遗传性出血性疾病，也可作为一种获得性综合征（AVWS）出现。这些疾病是由于血浆蛋白血管性血友病因子（VWF）的缺陷和/或缺乏而产生的。对这些与VWF相关的疾病进行实验室检测需要评估VWF水平和VWF活性，由于VWF为预防出血发挥多种功能，因此对VWF活性的评估需要进行多种检测。本文描述了几种通过VWF瑞斯托霉素辅因子（VWF:RCo）评估VWF活性的方法。这些检测通过定量评估VWF蛋白与血小板或其他颗粒的黏附情况，以及随后在加入瑞斯托霉素的促进下检测黏附的VWF来确定VWF活性。最常用的VWF:RCo检测方法包括血小板凝集试验、乳胶凝集试验和化学发光免疫分析（CLIA），本章将对其中三种方法进行描述。
