Unit 770, INSERM, Le Kremlin-Bicêtre, France; UMR_S 770, Univ Paris-Sud, Le Kremlin-Bicêtre, France.
J Thromb Haemost. 2013 Jun;11 Suppl 1:202-11. doi: 10.1111/jth.12226.
Quantitative deficiencies in von Willebrand factor (VWF) are associated with abnormal hemostasis that can manifest in bleeding or thrombotic complications. Consequently, many studies have endeavored to elucidate the mechanisms underlying the regulation of VWF plasma levels. This review focuses on the role of VWF clearance pathways. A summary of recent developments are provided, including results from genetic studies, the relationship between glycosylation and VWF clearance, the contribution of increased VWF clearance to the pathogenesis of von Willebrand disease and the identification of VWF clearance receptors. These different studies converge in their conclusion that VWF clearance is a complex phenomenon that involves multiple mechanisms. Deciphering how such different mechanisms coordinate their role in this process is but one of the remaining challenges. Nevertheless, a better insight into the complex clearance pathways of VWF may help us to better understand the clinical implications of aberrant clearance in the pathogenesis of von Willebrand disease and perhaps other disorders as well as aid in developing alternative therapeutic approaches.
血管性血友病因子（VWF）的定量缺乏与异常止血有关，可表现为出血或血栓并发症。因此，许多研究都致力于阐明 VWF 血浆水平调节的机制。本综述重点介绍 VWF 清除途径的作用。提供了最近研究结果的总结，包括遗传研究的结果、糖基化与 VWF 清除的关系、VWF 清除增加对血管性血友病发病机制的贡献以及 VWF 清除受体的鉴定。这些不同的研究都得出结论，VWF 的清除是一种复杂的现象，涉及多种机制。阐明这些不同的机制如何协调它们在这个过程中的作用是仍然存在的挑战之一。然而，更好地了解 VWF 的复杂清除途径可能有助于我们更好地理解血管性血友病发病机制中异常清除的临床意义，也许还有其他疾病，并有助于开发替代治疗方法。
