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血管性血友病因子:出血与血栓形成的十字路口。

von Willebrand factor: at the crossroads of bleeding and thrombosis.

机构信息

INSERM U770, 80 rue du General Leclerc, 94276 Le Kremlin-Bicêtre, France.

出版信息

Int J Hematol. 2012 Apr;95(4):353-61. doi: 10.1007/s12185-012-1041-x. Epub 2012 Apr 5.

Abstract

Hemostasis and thrombosis represent two sides of the same coin. Hemostasis maintains blood fluidity in the vascular system while allowing for rapid thrombus formation to prevent excessive hemorrhage after blood vessel injury. Thrombosis is a pathologic extension of the normal hemostatic mechanism, occurring when unwanted clot formation develops in certain pathological situations. The molecular mechanisms underlying both phenomena are fundamentally identical. One of the key players in both processes is the plasma glycoprotein von Willebrand factor, which perfectly illustrates this duality between hemostatic and thrombotic mechanisms. The purpose of this review is to discuss novel findings on the role of von Willebrand factor at this interface, and how some of these findings may help develop new therapeutic strategies.

摘要

止血和血栓形成代表同一枚硬币的两面。止血在血管系统中保持血液流动性,同时允许在血管损伤后迅速形成血栓以防止过度出血。血栓形成是正常止血机制的病理性延伸,当在某些病理情况下发生不需要的血栓形成时发生。这两种现象的分子机制在根本上是相同的。两种过程中的关键参与者之一是血浆糖蛋白血管性血友病因子,它完美地说明了止血和血栓形成机制之间的这种双重性。本文的目的是讨论在这一界面上血管性血友病因子作用的新发现,以及这些发现如何帮助开发新的治疗策略。

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