血管性血友病(von Willebrand disease,VWD)是临床上最常见的一种遗传性出血性疾病,其发病机制是患者的血管性血友病因子(von Willebrand factor,VWF)基因突变,导致血浆VWF数量减少或质量异常。国外报道VWD发病率为0·1/万至1/万[1]。我国尚无统计学资料。我院近年来已发现130 Von Willebrand disease (VWD) is the most common clinical hereditary bleeding disease, the pathogenesis of which is the von Willebrand factor (VWF) gene mutation in patients, leading to plasma VWF reduce the number of or abnormal quality. Foreign reports of the incidence of VWD 0.1 / million to 1 / 10,000 [1]. There is no statistical data in our country. Our hospital has found 130 in recent years