Mannucci P M, Abildgaard C F, Gralnick H R, Hill F G, Hoyer L W, Lombardi R, Nilsson I M, Tuddenham E, Meyer D
Thromb Haemost. 1985 Dec 17;54(4):873-6.
A multicenter study of various types of von Willebrand's disease (vWD) was conducted in order to compare the different electrophoretic techniques used to evaluate von Willebrand factor multimers in plasma. Seven laboratories participated in the blind study of eight plasma samples from two healthy subjects and six vWD types and subtypes (Ia, Ib, IIA, IIB, IIC and IID). From the results of the multimeric analysis of these samples, it appears that the differential diagnosis of vWD types and subtypes should be first approached by using a low-resolution electrophoretic technique, where each vWF multimer appears as a single band. Low-resolution techniques differentiate type I from type II, subtype Ia from Ib and also subtype IIA from other type II subtypes. When type II subtypes other than IIA are identified with these techniques, samples should be rerun using high resolution techniques that resolve each of the fastest migrating multimers in at least three subbands, and permit the differentiation of subtypes IIB, IIC and IID.
为比较用于评估血浆中血管性血友病因子多聚体的不同电泳技术，开展了一项关于各类血管性血友病（vWD）的多中心研究。七个实验室参与了对来自两名健康受试者以及六种vWD类型和亚型（Ia、Ib、IIA、IIB、IIC和IID）的八个血浆样本的盲法研究。从这些样本的多聚体分析结果来看，vWD类型和亚型的鉴别诊断首先应采用低分辨率电泳技术，在该技术中每个血管性血友病因子（vWF）多聚体呈现为一条单带。低分辨率技术可区分I型和II型、Ia亚型和Ib亚型，以及IIA型和其他II型亚型。当使用这些技术识别出除IIA之外的II型亚型时，样本应重新采用高分辨率技术进行检测，高分辨率技术可将每个迁移最快的多聚体解析为至少三个亚带，并能区分IIB、IIC和IID亚型。
