Pescatore P, Horellou H M, Conard J, Piffoux M, Van Dreden P, Ruskone-Fourmestraux A, Samama M
Laboratoire Central d'Hématologie, Hôtel-Dieu, Paris, France.
Thromb Haemost. 1993 Apr 1;69(4):311-5.
We describe a 57-year-old woman with homozygous protein C deficiency and mild thrombotic manifestations consisting of three spontaneous distal deep vein thromboses occurring after the age of 45. Previous surgery and pregnancies had been uneventful. Low but detectable protein C antigen and activity levels (both 20%) were discovered on the occasion of skin necrosis induced by oral anticoagulation. This therapy was interrupted because of skin necrosis and several episodes of disseminated intravascular coagulation (DIC) at the initiation of treatment despite a cautious protocol. No recurrent thromboembolic event has occurred in our patient using prophylactic doses of low molecular weight heparin for 24 months. New therapeutic approaches might be the administration of low molecular weight heparin or oral anticoagulation associated with protein C replacement in the induction period. This case reflects the variability of expression of protein C deficiency as well as the potential hazards of antivitamin K anticoagulation in this disorder.
我们描述了一位57岁的女性，她患有纯合子蛋白C缺乏症，并有轻度血栓形成表现，包括45岁以后发生的3次自发性远端深静脉血栓形成。既往手术和妊娠均顺利。在口服抗凝药引起皮肤坏死时，发现蛋白C抗原和活性水平较低但可检测到（均为20%）。尽管治疗方案谨慎，但由于皮肤坏死以及治疗开始时出现的几次弥散性血管内凝血（DIC）发作，该治疗被中断。我们的患者使用预防剂量的低分子量肝素24个月，未发生复发性血栓栓塞事件。新的治疗方法可能是在诱导期给予低分子量肝素或口服抗凝药并补充蛋白C。该病例反映了蛋白C缺乏症表达的变异性以及抗维生素K抗凝在该疾病中的潜在风险。
