血管性假血友病研究进展

血管性假血友病，亦称VonWi！lebrandDisease（简写vWD），是最常见的遗传性出血性疾病，首先由WillebrandE。V于1926年描述’‘’，临床上很难与血小板或凝血因子异常性出血疾病鉴别，对VWD的诊断和治疗，一直是临床医生较棘手的难题。近几年对yWD在分型、诊断和治疗方面进行分于生物学研究，有了新的进展。综述于下：一、VWD的分子生物学研究及分型vWD的病因是VonWillebrand因于（VWF）异常，有的类型同时伴有因子W促凝活性（皿：C）血浆水平的降低，导致临床上皮肤，粘膜出血为特征的出血性疾病。VWD应注意同血友病甲区别。…

ADVANCES IN THE STUDIES OF VON WILLEBRAND DISEASE

The Von Willebrand Disease(vWD) is the most common haemorrhagic disease of genetics, it is very difficult to diagnose and treat of vWD in clinical. This paper summed up the development in classifying types and diagnosis of the vWD by applying molecular biologic techniques, in recent years. It is indicated that classification of the vWD plays an important part in the treatment of vWD.