Klingemann H G, Broekmans A W, Bertina R M, Egbring R, Loeliger E A
Klin Wochenschr. 1984 Oct 15;62(20):975-8. doi: 10.1007/BF01728427.
Protein C is a vitamin K-dependent protein which is produced in the liver. Activated protein C has an anticoagulant effect by inactivating the clotting factors V and VIII. We report on a young female patient who suffered from recurrent thrombosis of the deep calf and pelvic veins with pulmonary embolism. Superficial thrombophlebitis also occurred repeatedly. In the plasma of this patient we found reduced levels of protein C antigen (0.62 U/ml) and activity (0.42 U/ml). Investigation of other family members revealed a protein C deficiency in her father, sister, and son. During the anticoagulant treatment with Marcumar the patient developed a coumarin-induced skin necrosis, to which complication a protein C deficiency is evidently predisposed. This complication could only be prevented if heparin was given simultaneously during the adjustment period.
蛋白C是一种在肝脏中产生的维生素K依赖蛋白。活化蛋白C通过使凝血因子V和VIII失活而具有抗凝作用。我们报告了一名年轻女性患者，她反复出现小腿深部和盆腔静脉血栓形成并伴有肺栓塞。浅表血栓性静脉炎也反复发生。在该患者的血浆中，我们发现蛋白C抗原水平降低（0.62 U/ml）和活性降低（0.42 U/ml）。对其他家庭成员的调查显示，她的父亲、姐姐和儿子存在蛋白C缺乏。在用苯丙香豆素进行抗凝治疗期间，该患者发生了香豆素诱导的皮肤坏死，蛋白C缺乏显然易导致这种并发症。只有在调整期同时给予肝素，才能预防这种并发症。
