Franchini Massimo, Mannucci Pier Mannuccio
Immunohematology and Transfusion Centre, Department of Pathology and Laboratory Medicine, University Hospital of Parma, Italy.
Semin Thromb Hemost. 2008 Oct;34(7):663-9. doi: 10.1055/s-0028-1104545. Epub 2008 Dec 15.
von Willebrand factor (VWF) is a multimeric plasma protein that mediates platelet adhesion and aggregation at sites of vascular injury and also acts as a protective carrier of factor VIII. Although the acquired or inherited deficiency of VWF is associated with a bleeding tendency, there is increasing evidence that VWF also plays a pivotal role in thrombosis. The presence in plasma of unusually large VWF multimers, due to the congenital or acquired deficiency of the VWF-cleaving metalloproteinase ADAMTS13, has been implicated in the pathogenesis of thrombotic thrombocytopenic purpura. In addition, high plasma levels of VWF have been associated with an increased risk of atherothrombosis. The current article reviews the literature and provides evidence for the two-faced character that VWF therefore represents.
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，同时还作为凝血因子VIII的保护性载体。尽管VWF的获得性或遗传性缺乏与出血倾向有关，但越来越多的证据表明，VWF在血栓形成中也起着关键作用。由于VWF裂解金属蛋白酶ADAMTS13先天性或获得性缺乏，血浆中出现异常大的VWF多聚体，这与血栓性血小板减少性紫癜的发病机制有关。此外，血浆中VWF水平升高与动脉粥样硬化血栓形成风险增加有关。本文回顾了相关文献，并为VWF所代表的两面性特征提供了证据。
