Odegaard O R, Try K, Ly B, Gundersen T, Linnebo S
Sentrallaboratoriet, Aker sykehus, Oslo.
Tidsskr Nor Laegeforen. 1993 Nov 20;113(28):3460-3.
Protein S deficiency increases risk of thrombosis. At present, we have information on 63 Norwegian individuals with hereditary protein S deficiency belonging to 25 different families. 42 of the individuals have experienced at least one thromboembolic episode, and seven a cerebral infarction before the age of 70 years. The amount of free protein S in plasma is dependent on variation of the acute phase protein C4b-binding protein (C4bBP). Acute phase response with increased C4bBP induces free protein S deficiency, and increases risk of thrombosis. In patients with protein S deficiency, warfarin may reduce free protein S to critically low levels, and thus explain why, in some patients, recurrent thrombosis occurs during warfarin treatment. In this situation, warfarin should be replaced by heparin.
蛋白S缺乏会增加血栓形成的风险。目前,我们掌握了63名挪威遗传性蛋白S缺乏个体的信息,他们分属于25个不同的家族。其中42人经历过至少一次血栓栓塞事件,7人在70岁之前发生过脑梗死。血浆中游离蛋白S的含量取决于急性期蛋白C4b结合蛋白(C4bBP)的变化。C4bBP增加的急性期反应会导致游离蛋白S缺乏,并增加血栓形成的风险。在蛋白S缺乏的患者中,华法林可能会将游离蛋白S降低到极低水平,这就解释了为什么在一些患者中,华法林治疗期间会反复发生血栓形成。在这种情况下,应将华法林换成肝素。