先天性蛋白C 缺乏是一种常染色体显性遗传疾病,可分为完全性及部分性两种,前者为纯合子,多发生在新生儿时期,表现为暴发性紫癜.后者为杂合子,50%在成年期出现反复血栓.用血浆或蛋白C 浓缩剂对完全蛋白C 缺乏有效,抗凝剂对部分蛋白C 缺乏有效.现从三方面叙述如下. Congenital protein C deficiency is an autosomal dominant genetic disease can be divided into two kinds of complete and partial, the former is homozygote, occurred in neonatal period, the performance of fulminant purpura. The latter is heterozygous, 50% repeated thrombosis in adulthood with plasma or protein C concentrates on the complete lack of protein C, anticoagulants on the part of the lack of protein C. Now described from the following three aspects.