Asbeck F, Meyer-Boernecke D, van de Loo J
Haemostasis. 1977;6(5):303-9. doi: 10.1159/000214195.
Suppression of the fibrinolytic system is a well-known phenomenon in patients with Behçet's disease. It is generally explained by an increase in the inhibitory potential. In order to prove this theory, the fibrinolytic system was investigated in 5 male patients with severe chronic Behçet's disease. By the venous occlusion test, a marked suppression of the activatability of the system could be demonstrated (euglobulin lysis time, various fibrin plate assays). Results of analysis of the known inhibitors could not explain the phenomenon: antithrombin III, alpha2-macroglobulin, fast-reacting alpha2-antiplasmin, C1 inactivator, and the plasmin-antiplasmin complex were normal. The only change was an elevation of alpha1-antitrypsin and fibrinogen in 4 patients possibly due to an acute phase reaction. In addition to the possibility of a hitherto unidentified inhibitor, therefore, a decrease in production or in release of vessel wall activators must also be considered in the pathogenesis of this disorder.
纤维蛋白溶解系统的抑制在白塞病患者中是一种众所周知的现象。通常认为这是由于抑制潜能增加所致。为了证实这一理论,对5例重症慢性白塞病男性患者的纤维蛋白溶解系统进行了研究。通过静脉闭塞试验,可以证明该系统的激活能力受到明显抑制(优球蛋白溶解时间、各种纤维蛋白平板试验)。已知抑制剂的分析结果无法解释这一现象:抗凝血酶III、α2-巨球蛋白、快速反应性α2-抗纤溶酶、C1灭活剂和纤溶酶-抗纤溶酶复合物均正常。唯一的变化是4例患者的α1-抗胰蛋白酶和纤维蛋白原升高,这可能是由于急性期反应所致。因此,除了可能存在一种迄今尚未鉴定的抑制剂外,在这种疾病的发病机制中还必须考虑血管壁激活剂的产生或释放减少。