血友病甲(以下简称血友病)及血管性假血友病是二种最常见的遗传性出血性疾病。很久以来已经明确血友病是由于缺乏抗血友病球蛋白(AHG)或因子VIII所致,而血管性假血友病则是由于缺乏Von Willebrand因子(VWF)所引起的。但对因子VIII的本质,这二种遗传性出血疾病的临床变异,实验室检查方法尤其是携带者的检 Hemophilia A (hereinafter referred to as hemophilia) and von Willebrand disease are the two most common hereditary hemorrhagic diseases. It has long been clear that hemophilia is due to the lack of antihemophilic globulin (AHG) or factor VIII, whereas von Willebrand disease is due to a lack of Von Willebrand factor (VWF). But for the nature of Factor VIII, clinical variants of these two hereditary bleeding disorders, laboratory tests, especially those of carriers