Federici Augusto B, Canciani Maria T
Haematologica. 2009 May;94(5):610-5. doi: 10.3324/haematol.2009.005751.
von Willebrand disease (VWD) is the most common inherited bleeding disorder. In this perspective article, Drs. Federici and Canciani review how correlating the clinical, laboratory and genetic features of von Willebrand disease has led to improved understanding of its pathophysiology. They show how this has rationalized classification, which in turn can be used to improve treatment of these patients.
血管性血友病(VWD)是最常见的遗传性出血性疾病。在这篇观点文章中,费代里西博士和坎恰尼博士回顾了将血管性血友病的临床、实验室和基因特征相互关联是如何增进对其病理生理学的理解的。他们展示了这如何使分类合理化,而分类反过来又可用于改善这些患者的治疗。