Department of Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Westmead NSW, Australia.
Sydney Centres for Thrombosis and Haemostasis, Westmead Hospital, Westmead NSW, Australia.
Hamostaseologie. 2020 Nov;40(4):431-442. doi: 10.1055/s-0040-1713735. Epub 2020 Jun 26.
von Willebrand factor (VWF) represents a large and complex adhesive plasma protein whose main function is to provide a bridge between blood platelets and damaged endothelium, and thus facilitate primary hemostasis. VWF also binds to FVIII, preventing early proteolysis, and delivers this cargo to sites of vascular injury, thereby promoting clot formation and secondary hemostasis. An absence, deficiency, or defect in VWF can lead to a bleeding diathesis called von Willebrand disease (VWD), considered the most common inherited bleeding disorder. Contemporary laboratory assays used in VWD diagnosis/exclusion comprise a myriad of assays that identify the quantity (level) of VWF, as well as the multitude of VWF activities. These may use the following test abbreviations: VWF:Ag, VWF:RCo, VWF:CB, VWF:GPIbR, VWF:GPIbM, VWF:FVIIB, VWF:Ab. The current review explains what these assays are, as well as their place in VWD diagnostics.
血管性血友病因子(VWF)是一种大型且复杂的黏附性血浆蛋白,其主要功能是在血小板和受损的内皮细胞之间架起桥梁,从而促进初步止血。VWF 还与 FVIII 结合,防止其过早被蛋白水解,并将其运送到血管损伤部位,从而促进血栓形成和二次止血。VWF 的缺失、缺乏或缺陷会导致一种称为血管性血友病(VWD)的出血倾向,这被认为是最常见的遗传性出血性疾病。用于 VWD 诊断/排除的当代实验室检测包括多种检测方法,可确定 VWF 的数量(水平)以及多种 VWF 活性。这些可能使用以下测试缩写:VWF:Ag、VWF:RCo、VWF:CB、VWF:GPIbR、VWF:GPIbM、VWF:FVIIB、VWF:Ab。本综述解释了这些检测方法及其在 VWD 诊断中的作用。