阵发性睡眠性血红蛋白尿症的血栓形成研究进展

阵发性睡眠性血红蛋白尿症(PNH)引发的补体系统持续活化、血管内溶血与凝血系统相作用，使得PNH处于血栓的高危状态中，是患者致死和致残的主要原因。非常见部位血栓，血栓合并溶血或血细胞减少等情况需要行PNH筛查。血栓形成可能与血小板活化、精氨酸酶释放消耗NO，以及溶血释放的促凝物质有关。低分子肝素溶栓和随后使用维生素K依赖性凝性因子拮抗剂预防是常用措施。血栓患者行异基因造血干细胞移植不能有效延长生存期，而eculizumab能显著控制血栓形成。

Research progress of thrombosis paroxysmal nocturnal hemoglobinuria

Abstract：The complement and coagulation systems are closely integrated with each influencing the activity of platelet in paroxysmal nocturnal hemoglobinuria(PNH).Thromboembolism is the most common cause of mortality and morbidity in patients with PNH.When thrombosis in an unusual site,or with evidence of hemolysis,or any cytopenia,testing for PNH by flow cytometry should be done.Activated platelets,release of arginase resulting in NO depletion,release of procoagulant red cell microparticles are related with thrombosis.Low-molecular-weight heparin,and continuing anticoagulation with the vitamin K antagonists is generally recommended.Allogeneic bone marrow transplant has been previously considered but could not improve the survival rate.Eculizumab treatment resulted in a reduction in the thromboembolism events effectively.