凝血因子Ⅷ(FⅧ)缺乏症是一种十分罕见的出血性疾病,发病率仅为1/2 000 000,占出血性疾病的6%[1],可分为遗传性和获得性两种类型。遗传性F缺乏症为一种常染色体隐性遗传病,主要是由于FⅧ-A和(或)FⅧ-B基因的缺陷所致,发病率约为1/3 000 000,目前全世界大约有500例相关报道[2],而获得性FⅧ缺乏症国际上有正式文献报道的病例有38例。遗传性FⅧ缺乏症临床主要表现为反 FⅧ deficiency is a very rare hemorrhagic disease with a prevalence of only 1/2 000 000, accounting for 6% of hemorrhagic diseases [1] and can be divided into two types, hereditary and acquired . Hereditary F deficiency is an autosomal recessive disease, mainly due to defects in F Ⅷ -A and / or F Ⅷ -B gene, the incidence of about 1 / 3,000,000, the world now has about 500 cases of related reports [2], and acquired FⅧ deficiency in the world there are 38 cases of formal reports in the literature. Hereditary FⅧ deficiency clinical manifestations of the main anti