Franchini Massimo, Lippi Giuseppe
Servizio di Immunoematologia e Trasfusione, Azienda Ospedaliera di Verona, Verona, Italy.
Crit Rev Clin Lab Sci. 2007;44(2):115-49. doi: 10.1080/10408360600966753.
von Willebrand factor (VWF) is a multimeric plasma protein that mediates platelet adhesion as well as platelet aggregation at sites of vascular injury and acts as a carrier of factor VIII. Although acquired or inherited VWF deficiency is associated with a bleeding tendency, there is increasing evidence that VWF has a pivotal role in thrombogenesis. In fact, while the presence in the plasma of unusually large VWF multimers, due to a congenital or acquired deficiency of a VWF-cleaving metalloprotease, has been implicated in the pathogenesis of thrombotic thrombocytopenic purpura, high plasma levels of VWF have been associated with an increased risk of both arterial and venous thrombosis. The role of VWF in normal and pathological hemostasis is discussed in this review, and important advances in the pathophysiology, diagnosis, and treatment of VWF-associated disorders are also described.
血管性血友病因子（VWF）是一种多聚体血浆蛋白，它在血管损伤部位介导血小板黏附和聚集，并作为因子VIII的载体。尽管获得性或遗传性VWF缺乏与出血倾向相关，但越来越多的证据表明VWF在血栓形成中起关键作用。事实上，由于VWF裂解金属蛋白酶先天性或获得性缺乏导致血浆中存在异常大的VWF多聚体，这与血栓性血小板减少性紫癜的发病机制有关，而血浆中VWF水平升高与动脉和静脉血栓形成风险增加有关。本文综述了VWF在正常和病理性止血中的作用，并描述了VWF相关疾病在病理生理学、诊断和治疗方面的重要进展。
