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xue guan xing xue you bing zhen duan yu zhi liao zhong guo zhuan jia gong shi (2012 nian ban )
Pages: 980-981
Year: Issue:  11
Journal: Chinese Journal of Hematology

Abstract: 一、概述 血管性血友病(von Willebrand discase,VWD)是最常见的遗传性出血性疾病,患者von Willebrand因子(von Willebrand factor,VWF)基因突变导致血浆VWF数量减少或质量异常. VWF由血管内皮细胞与巨核细胞合成.VWF的主要作用:①与血小板膜糖蛋白(GP)Ⅰ b-Ⅸ-V复合物及内皮下胶原结合,介导血小板黏附至血管损伤部位;②作为因子Ⅷ(FⅧ)的载体,具有稳定FⅧ的作用.根据VWD发病机制,VWD可分为三种类型:1型为VWF量的减;3型为VWF量显著减少或缺如;2型为VWF质的缺陷,又可分为2A型缺乏VWF高分子多聚物,血小板黏附活性降低;2B型VWF与GPⅠ b亲和力增加,导致高分子多聚物减少;2M型血小板黏附活性降低,但VWF多聚物正常;2N型VWF与FⅧ亲和力降低,导致FⅧ活性显著减少.
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