蛋白C(PC)是体内血栓、抗凝调节系统即PC途径的中心蛋白,具有潜在的抗凝和纤溶活性。遗传性PC缺乏症(HPCD)是一种常染色体遗传病,分杂合型和纯合型两类,前者以成人的复发性静脉血栓形成为特征,后者发病年龄小,并为新生儿暴发性紫癜的常见病因。近年来本病愈来愈受到国内外学者的普遍重视,本文就其遗传分型、诊断及治疗近况综述如下。 Protein C (PC) is a central protein of the thrombus, anticoagulant regulatory system, the PC pathway, with potential anticoagulant and fibrinolytic activity. Hereditary PC deficiency (HPCD) is an autosomal genetic disorder, heterozygous and homozygous two types, the former is characterized by recurrent venous thrombosis in adults, the latter age of onset is small, and the outbreak of newborns Purpura common causes. In recent years, the disease more and more widespread attention by scholars at home and abroad, this article on its genetic typing, diagnosis and treatment of the status are summarized below.