摘要阵发性睡眠性血红蛋白尿症(PNH)、镰状细胞性贫血等溶血性疾病患者发生血管内溶血时,体内可产生大量的游离血红蛋白(FHb),并且释放细胞内精氨酸酶.前者可与一氧化氮(NO)迅速结合,后者则可降解NO的合成底物L-精氨酸,加之溶血后活性氧簇(ROS)增加等因素,共同导致溶血性疾病患者体内的NO耗竭,并且进一步引发血管内皮损伤和微循环血管舒缩障碍,最终导致多器官的急、慢性损伤.近年,随着对血管内溶血过程中NO消耗机制的深入研究,相关疾病的常见病理损伤机制得以阐明,为NO的替代治疗开辟了一个崭新的途径.基于此,笔者拟就NO消耗机制及其替代治疗在血管内溶血性疾病中的研究进展进行综述.

abstractsIn the case of intravascular hemolysis in patients with hemolytic diseases such as paroxysmal nocturnal hemoglobinuria (PNH)and sickle cell disease (SCD),patients' body can produce a large amount of free hemoglobin (FHb)and release intracellular arginase.FHb can rapidly scavenge nitric oxide (NO)and arginase can degrade L-arginine,which is substrate for NO synthesis.All together with the increased amount of reactive oxygen species (ROS)after hemolysis,NO appears to be depleted in the circulation,which results damages to endothelial and dysfunction of microcirculation,as well as eventually lead to acute and chronic impair to multiple organs.In recent years,with the in-depth study of the mechanisms beneath NO depletion in intravascular hemolytic disorders, the common pathological mechanisms of such diseases have been elucidated,and NO replacement therapy seems to be an all new approach.In view of this,this article reviews the research progress in the field of NO depletion and its replacement therapy in intravascular hemolytic diseases.