Lund University, Department of Hematology and Coagulation Disorders Skåne University Hospital, Malmö, Sweden.
Haemophilia. 2012 Sep;18 Suppl 6(0 6):1-13. doi: 10.1111/j.1365-2516.2012.02925.x.
von Willebrand's disease (VWD) is probably the most common bleeding disorder, with some studies indicating that up to 1% of the population may have the condition. Over recent years interest in VWD has fallen compared to that of haemophilia, partly the result of focus on blood-borne diseases such as HIV and hepatitis. Now the time has come to revisit VWD, and in view of this some 60 international physicians with clinical and scientific interest in VWD met over 4 days in 2010 in the Åland islands to discuss state-of-the-art issues in the disease. The Åland islands are where Erik von Willebrand had first observed a bleeding disorder in a number of members of a family from Föglö, and 2010 was also the 140th anniversary of his birth. This report summarizes the main papers presented at the symposium; topics ranged from genetics and biochemistry through to classification of VWD, pharmacokinetics and laboratory assays used in the diagnosis of the disease, inhibitors, treatment guidelines in different age groups including the elderly who often have comorbid conditions that present challenges, and prophylaxis. Other topics included managing surgeries in patients with VWD and the role of FVIII in VWF replacement, a controversial subject.
血管性血友病（von Willebrand's disease，VWD）可能是最常见的出血性疾病，一些研究表明，多达 1%的人口可能患有这种疾病。近年来，与血友病相比，人们对 VWD 的兴趣有所下降，部分原因是人们关注了艾滋病毒和肝炎等血液传播疾病。现在是重新审视 VWD 的时候了，考虑到这一点，2010 年，约 60 名对 VWD 具有临床和科学兴趣的国际医生在 Åland 群岛会面了 4 天，讨论了该疾病的最新问题。Åland 群岛是 Erik von Willebrand 首次在来自 Föglö 的一些家族成员中观察到出血性疾病的地方，2010 年也是他诞辰 140 周年。本报告总结了专题讨论会上提交的主要论文；议题范围从遗传学和生物化学到 VWD 的分类、疾病诊断中使用的药代动力学和实验室检测、抑制剂、不同年龄段的治疗指南，包括经常患有合并症的老年人，这些合并症带来了挑战，以及预防措施。其他议题包括 VWD 患者手术的管理以及 FVIII 在 VWF 替代中的作用，这是一个有争议的话题。
