本病也称 Von Willebranel 病(VWD),是一种较常见的遗传性出血性疾病。它是由于 Von Willebrand因子(VWF)血浆浓度异常,分子结构或功能异常引起的。近年来,国外对 VWF 的分子生物学及 VWD 的分类进行了大量研究。本文就 VWF 的分子生物学、VWD 的分类、临床表现、诊断和治疗进展作一综述。 This disease, also known as Von Willebranel disease (VWD), is a more common form of hereditary hemorrhagic disease. It is caused by an abnormal plasma concentration of Von Willebrand Factor (VWF), molecular structure or dysfunction. In recent years, a large number of studies abroad have been conducted on the molecular biology of VWF and the classification of VWD. This article reviews the molecular biology of VWF, classification of VWD, clinical manifestations, diagnosis and treatment progress.