血管性血友病：内科医师指南。
von Willebrand disease: A guide for the internist.
机构信息
Associate Professor, Department of Internal Medicine, Division of Hematology and Oncology, University of Virginia, Charlottesville, VA
Department of Internal Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA.
出版信息
Cleve Clin J Med. 2024 Feb 2;91(2):119-127. doi: 10.3949/ccjm.91a.22033.
von Willebrand disease (VWD), the most common inherited bleeding disorder, results when patients either do not make enough von Willebrand factor (VWF) or make defective VWF. The pathophysiology of this disorder is complex but needs to be understood to interpret the diagnostic tests. Most patients have mild to moderate symptoms and can be adequately counseled and managed by a general internist, but some need to consult a hematologist. We review the pathophysiology of VWD, its subtypes, common presentations of each subtype, diagnostic testing, and management of mild as well as severe clinical manifestations of VWD.
血管性血友病（von Willebrand disease，VWD）是最常见的遗传性出血性疾病，当患者要么无法产生足够的血管性血友病因子（von Willebrand factor，VWF），要么产生缺陷型 VWF 时，就会发生 VWD。这种疾病的病理生理学较为复杂，但需要了解这些知识才能解读诊断性检测。大多数患者的症状为轻至中度，可由普通内科医生进行充分的咨询和管理，但有些患者需要咨询血液科医生。我们回顾了 VWD 的病理生理学、亚型、每种亚型的常见表现、诊断性检测，以及 VWD 轻度和重度临床表现的管理。
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