Schmitz-Huebner U, Knop J
Thromb Res. 1984 May 15;34(4):277-85. doi: 10.1016/0049-3848(84)90384-0.
Suppression of the fibrinolytic system is a well-known phenomenon in patients with Behcet's disease regardless of whether they present thrombotic complications. This finding has been related to impaired production and/or release of plasminogen activators from the vascular endothelium. In previous studies a diminished release of PF4 upon heparin stimuli was observed in plasma from patients with Behcet's disease and interpreted as an additional indicator for endothelial cell dysfunction. In the present investigations, 12 patients and 10 healthy volunteers received DDAVP infusions and euglobulin clot lysis time, factor VIII activities and 6-keto-PGF1 alpha levels in plasma were repeatedly determined before and after infusions. At different times following DDAVP infusion, euglobulin clot lysis time was significantly longer and levels of F.VIII R:Ag were lower in patients than in normals. F. VIII:C activity increased in both groups, whereas no changes were seen in the plasma levels of 6-Keto-PGF1 alpha either in normals or in patients. It is concluded that the disseminated damage of endothelial tissue associated with Behçet's disease correlates with multiple endothelial cell dysfunctions and subsequent hemostatic abnormalities.
无论是否出现血栓并发症，白塞病患者体内纤溶系统受抑制都是一种众所周知的现象。这一发现与血管内皮细胞纤溶酶原激活物生成和/或释放受损有关。在先前的研究中，观察到白塞病患者血浆中肝素刺激后PF4释放减少，并将其解释为内皮细胞功能障碍的另一个指标。在本研究中，12例患者和10名健康志愿者接受了去氨加压素输注，并在输注前后反复测定血浆中优球蛋白凝块溶解时间、因子VIII活性和6-酮-PGF1α水平。在去氨加压素输注后的不同时间，患者的优球蛋白凝块溶解时间明显长于正常人，F.VIII R:Ag水平低于正常人。两组的F.VIII:C活性均升高，而正常人或患者血浆中6-酮-PGF1α水平均无变化。结论是，与白塞病相关的内皮组织弥漫性损伤与多种内皮细胞功能障碍及随后的止血异常相关。
