Lobato-Mendizábal E, Ruiz-Argüelles G J
Hospital Universitario de Puebla.
Rev Invest Clin. 1990 Jan-Mar;42(1):54-62.
In this review paper, the salient features of the anticoagulant/fibrinolytic mechanism depending on coagulation protein C, protein S and thrombomodulin are reviewed. Coagulation protein C, activated at the endothelial cell surface in the presence of the complex thrombin/thrombomodulin exerts two anti-thrombotic effects: one anticoagulant dependent on the free protein S and the other pro-fibrinolytic, independent of protein S. Both inherited and acquired deficiencies of protein C and/or protein S lead to a thrombosis-prone state that has to be identified promptly to avoid vaso-occlusive episodes. The experience in Mexico with both the identification and treatment of these deficiencies is reviewed; it is interesting that we have found that patients with autoimmune disorders, mainly systemic lupus erythematosus and primary anti-phospholipid syndrome, have acquired deficiencies of this anticoagulant mechanism that may be related to the thrombogenesis observed in these patients.
在这篇综述论文中,对依赖凝血蛋白C、蛋白S和血栓调节蛋白的抗凝/纤维蛋白溶解机制的显著特征进行了综述。凝血蛋白C在凝血酶/血栓调节蛋白复合物存在的情况下在内皮细胞表面被激活,发挥两种抗血栓作用:一种依赖游离蛋白S的抗凝作用,另一种不依赖蛋白S的促纤维蛋白溶解作用。蛋白C和/或蛋白S的遗传性和获得性缺乏都会导致易血栓形成状态,必须及时识别以避免血管闭塞性发作。本文回顾了墨西哥在这些缺乏症的识别和治疗方面的经验;有趣的是,我们发现自身免疫性疾病患者,主要是系统性红斑狼疮和原发性抗磷脂综合征患者,获得了这种抗凝机制的缺乏,这可能与这些患者中观察到的血栓形成有关。