遗传性蛋白Ｃ(ＰＣ)缺陷症 ,是以ＰＣ的活性 (ＰＣ∶Ａ)和抗原水平 (ＰＣ∶Ａｇ)低下为特征的常染色体显性遗传性疾病。本症以反复静脉血栓形成为主要表现。 1981年 ,Ｇｒｉｆｆｉｎ等报道了第 1个遗传性ＰＣ缺陷症家族 ,至 1995年已报道了 315例[1] ,但国内还未见报道。最近我 Hereditary protein C (PC) deficiency is an autosomal dominant disorder characterized by a decrease in PC activity (PC: A) and antigen level (PC: Ag). The disease with repeated venous thrombosis as the main performance. In 1981, Griffin et al. Reported the first hereditary family of PC-deficient cases. Up to 1995, 315 cases have been reported [1], but no reports have been reported in China. Recently I