Lemesle F G, Gris J C, Schwed J F, Arich C
Service de Réanimation, Hôpital Hoche, Nimes, France.
Intensive Care Med. 1993;19(2):115-6. doi: 10.1007/BF01708373.
A 32-year-old pregnant woman developed meningococcemia associated purpura fulminans and quickly improved with therapy. After this disease C4b-Binding Protein (C4bBP) plasma levels remained very low while protein S activity was in the normal range. Familial investigation proved a hereditary C4bBP deficiency. This observation points out the role of the protein C-protein S system during acquired purpura fulminans.
一名32岁的孕妇发生了与暴发性紫癜相关的脑膜炎球菌血症，经治疗后迅速好转。患此病后，C4b结合蛋白（C4bBP）的血浆水平一直很低，而蛋白S活性在正常范围内。家族调查证实存在遗传性C4bBP缺乏。这一观察结果指出了蛋白C-蛋白S系统在获得性暴发性紫癜中的作用。
