Pasi K J, Collins P W, Keeling D M, Brown S A, Cumming A M, Dolan G C, Hay C R M, Hill F G H, Laffan M, Peake I R
Department of Haematology, Barts and the London, Queen Mary's School of Medicine and Dentistry, London, UK.
Haemophilia. 2004 May;10(3):218-31. doi: 10.1111/j.1365-2516.2004.00886.x.
von Willebrand disease (VWD) is the commonest inherited bleeding disorder. The aim of therapy for VWD is to correct the two defects of haemostasis in this disorder, impaired primary haemostasis because of defective platelet adhesion and aggregation and impaired coagulation as a result of low levels of factor VIII. The objective of this guideline is to inform individuals making choices about the treatment and management of VWD including the use of therapeutic products. This is the second edition of this UK Haemophilia Centre Doctors' Organization (UKHCDO) guideline and supersedes the previous edition which was published in 1994.
血管性血友病（VWD）是最常见的遗传性出血性疾病。VWD的治疗目的是纠正该疾病中止血的两个缺陷，即由于血小板黏附和聚集缺陷导致的原发性止血受损，以及因因子VIII水平低而导致的凝血功能受损。本指南的目的是为那些对VWD的治疗和管理（包括使用治疗产品）做出选择的人提供信息。这是英国血友病中心医生组织（UKHCDO）本指南的第二版，取代了1994年出版的上一版。
