Hauert J, Tran-Thang C, Bachmann F
Schweiz Med Wochenschr. 1985 Dec 7;115(49):1773-5.
The multimeric composition of von Willebrand factor (vWF) in the plasma of 20 von Willebrand's disease patients was analyzed to determine the type of von Willebrand's disease and to evaluate retrospectively the predictive value of the classical tests. Analysis of the multimeric pattern revealed that 3 had type II and the other 17 type I von Willebrand's disease. The classical tests, especially crossed immunoelectrophoresis of vWF and the ristocetin-induced platelet agglutination test, did not permit correct classification but led to overestimation of the type II von Willebrand variant. Multimeric analysis of von Willebrand factor is necessary to diagnose von Willebrand's disease type II, in which DDAVP infusions should not be given since they are ineffective or may cause thrombocytopenia.
分析了20例血管性血友病患者血浆中血管性血友病因子（vWF）的多聚体组成，以确定血管性血友病的类型，并回顾性评估经典检测方法的预测价值。多聚体模式分析显示，3例为Ⅱ型血管性血友病，其余17例为Ⅰ型血管性血友病。经典检测方法，尤其是vWF的交叉免疫电泳和瑞斯托霉素诱导的血小板凝集试验，无法进行正确分类，反而导致对Ⅱ型血管性血友病变异型的高估。血管性血友病因子的多聚体分析对于诊断Ⅱ型血管性血友病是必要的，因为在Ⅱ型血管性血友病中，输注去氨加压素无效或可能导致血小板减少，不应给予输注。
