Franchini M, Mannucci P M
Pier Mannuccio Mannucci, MD, Scientific Direction, IRCCS Cà Granda Foundation Maggiore Policlinico Hospital, Via Pace 9, 20122 Milan, Italy, Tel.: + 39 02 55038377, Fax: + 39 02 50320723, E-mail:
Thromb Haemost. 2014 Sep 2;112(3):427-31. doi: 10.1160/TH13-11-0952. Epub 2014 Jun 5.
Von Willebrand disease (VWD), the most common genetic bleeding disorder, is characterised by a quantitative or qualitative defect of von Willebrand factor (VWF). Patients with VWD suffer from mucocutaneous bleeding, of severity usually proportional to the degree of VWF defect. In particular, gastrointestinal bleeding associated with angiodysplasia is often a severe symptom of difficult management. This review focuses on the pathophysiology, diagnosis and treatment of VWD-associated gastrointestinal angiodysplasia and related bleeding.
血管性血友病（VWD）是最常见的遗传性出血性疾病，其特征是血管性血友病因子（VWF）存在数量或质量缺陷。VWD患者会出现皮肤黏膜出血，严重程度通常与VWF缺陷程度成正比。特别是，与血管发育异常相关的胃肠道出血往往是一种难以处理的严重症状。本综述重点关注VWD相关胃肠道血管发育异常及相关出血的病理生理学、诊断和治疗。
