Favaloro Emmanuel J, Mohammed Soma
Haematology Department, Sydney Centres for Thrombosis and Haemostasis, Institute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, NSW Health Pathology, Westmead, NSW, 2145, Australia.
Haematology Department, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Westmead, NSW, 2145, Australia.
Methods Mol Biol. 2017;1646:417-433. doi: 10.1007/978-1-4939-7196-1_31.
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 the 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. This chapter describes several protocols for assessment of VWF activity by means of VWF collagen binding (VWF:CB). These assays identify VWF activity by quantitative assessment of VWF protein adhesion to collagen or collagen peptides and subsequent immunological detection of the adhered VWF. The most commonly performed assays for VWF:CB comprise enzyme-linked immunosorbent assays (ELISA) and chemiluminescent assay (CLIA), as 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VWF相关疾病的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，后者需要多种检测方法。本章介绍了几种通过VWF胶原结合（VWF:CB）评估VWF活性的方案。这些检测通过对VWF蛋白与胶原蛋白或胶原肽的粘附进行定量评估以及随后对粘附的VWF进行免疫检测来鉴定VWF活性。本章所述，最常用的VWF:CB检测方法包括酶联免疫吸附测定（ELISA）和化学发光测定（CLIA）。
