Miller J L, Boselli B D, Kupinski J M
Blood. 1984 Jan;63(1):226-30.
Previous studies performed in vitro have indicated that platelets from patients with platelet-type von Willebrand's disease (vWD) have receptors for von Willebrand factor (vWF) already exposed on their surfaces and that the addition of purified vWF or cryoprecipitate to patient platelet-rich plasma under stirring conditions is capable of inducing platelet aggregation and secretion. The present work reports the results of the transfusion of cryoprecipitate in a patient with platelet-type vWD. It is shown that, while factor VIII-related antigen and ristocetin cofactor activities maintain elevated levels for up to 12 hr following transfusion, the highest molecular weight vWF multimers decline rapidly. The platelet count also declines, followed in turn by a rise in the plasma level of platelet factor 4. Shortening of the bleeding time occurs only very transiently. The results of this study provide direct evidence that, in patients with platelet-type vWD, an abnormal interaction of their platelets with plasma vWF occurs in vivo, resulting in the absence of high molecular weight vWF multimers, low platelet counts, and impaired hemostasis that are characteristic of this disease.
以往的体外研究表明，血小板型血管性血友病（vWD）患者的血小板表面已暴露有血管性血友病因子（vWF）受体，并且在搅拌条件下向患者富含血小板的血浆中添加纯化的vWF或冷沉淀能够诱导血小板聚集和分泌。本研究报告了一名血小板型vWD患者输注冷沉淀的结果。结果显示，虽然输注后因子VIII相关抗原和瑞斯托霉素辅因子活性可维持升高水平长达12小时，但最高分子量的vWF多聚体迅速下降。血小板计数也下降，随后血浆中血小板因子4水平升高。出血时间仅短暂缩短。本研究结果提供了直接证据，表明在血小板型vWD患者中，其血小板与血浆vWF在体内发生异常相互作用，导致缺乏高分子量vWF多聚体、血小板计数低以及止血功能受损，这些都是该疾病的特征。
