Department of Endocrinology, Institute of Clinical Pathology and Medical Research, Westmead Hospital, New South Wales, Australia.
Thromb Haemost. 2012 Nov;108(5):999-1005. doi: 10.1160/TH12-05-0294. Epub 2012 Sep 26.
Deficiency of or defects in the plasma protein von Willebrand factor (VWF) lead to bleeding and von Willebrand disease (VWD), which may be congenital or acquired. VWD is considered the most common inherited bleeding disorder and laboratory testing for VWF level and activity is critical for appropriate diagnosis and management. We have designed and established a novel Flow Cytometry (FC) based method for measuring VWF antigen (VWF:Ag) and collagen binding (VWF:CB), together in the same tube and at the same time. The results of the novel FC method have been compared against existing reference methods using a range of normal and pathological material. Methods correlated well (VWF:Ag, r=0.866; VWF:CB, r=0.888) and generally permitted similar discrimination of quantitative versus qualitative VWD types (e.g. type 1 vs type 2A or 2B VWD). The novel procedure is expected to permit future streamlined performance of VWD screening, either using stand-alone FC systems or potentially incorporated into FC-capable automated blood cell and particle counters to allow for improved, automated and faster identification or exclusion of VWD.
血浆蛋白 von Willebrand 因子（VWF）的缺乏或缺陷会导致出血和血管性血友病（VWD），其可能是先天性的或获得性的。VWD 被认为是最常见的遗传性出血性疾病，VWF 水平和活性的实验室检测对于正确的诊断和管理至关重要。我们设计并建立了一种新型的基于流式细胞术（FC）的方法，用于测量 VWF 抗原（VWF：Ag）和胶原结合（VWF：CB），可在同一管中同时进行。使用一系列正常和病理材料，将新型 FC 方法的结果与现有参考方法进行了比较。方法相关性良好（VWF：Ag，r=0.866；VWF：CB，r=0.888），并且通常能够类似地区分定量和定性 VWD 类型（例如，1 型与 2A 或 2B VWD）。新型程序有望允许未来简化 VWD 筛查，无论是使用独立的 FC 系统还是潜在地整合到具有 FC 功能的自动血细胞和颗粒计数器中，以允许改进、自动化和更快地识别或排除 VWD。
