Unit of Coagulation Disorders, Department of Hematology and Clinical Chemistry Laboratory Services, Helsinki University Central Hospital, Helsinki, Finland.
Semin Thromb Hemost. 2011 Jul;37(5):495-502. doi: 10.1055/s-0031-1281034. Epub 2011 Nov 18.
Von Willebrand disease (VWD) is the most common inherited bleeding disorder characterized by spontaneous or tissue injury-related, mostly mucocutaneous, bleeding events. VWD affects both males and females and is caused by quantitative or qualitative deficiency of Von Willebrand factor. The diagnostic procedure is complicated because VWD is highly heterogeneous, and differential diagnosis from platelet disorders may be challenging. Moreover, these defects may even coexist, impacting the bleeding phenotype. Mild and moderate VWD can be difficult to distinguish from the normal population, and VWD subtyping may also be problematic. This article summarizes the guidelines of the Nordic Haemophilia Council (NHC), which are intended to serve as a practical tool and provide the standards for diagnosing and treating VWD patients. The complete Nordic Guidelines on VWD are available at the NHC Web site (http://nordhemophilia.org).
血管性血友病（VWD）是最常见的遗传性出血性疾病，其特征为自发性或与组织损伤相关的、主要为黏膜皮肤的出血事件。VWD 可影响男性和女性，是由血管性血友病因子的数量或质量缺乏引起的。由于 VWD 高度异质，且与血小板疾病的鉴别诊断可能具有挑战性，因此诊断程序较为复杂。此外，这些缺陷甚至可能共存，影响出血表型。轻度和中度 VWD 与正常人群难以区分，且 VWD 亚型的划分也可能存在问题。本文总结了北欧血友病理事会（NHC）的指南，旨在作为一种实用工具，为 VWD 患者的诊断和治疗提供标准。完整的北欧 VWD 指南可在 NHC 网站（http://nordhemophilia.org）上获得。
