红细胞(RBC)在溶血性贫血早期开始分解,可能是慢性的或危及生命的。在确定是否存在正常细胞或大细胞性贫血时应考虑。网状内皮系统的溶血可能发生在血管内,外血管,或者两者兼而有之。它涵盖了广泛的实验室和临床情况,生理和病理。只要红细胞分解的频率足够快,可以将血红蛋白水平降低到正常范围以下,发生溶血性贫血。微血管病性溶血性贫血(MAHA)是一个术语,用于描述由血管内RBC碎裂引起的非免疫性溶血,血管内RBC碎裂是由在外周循环中产生分裂细胞的微小动脉中的物质引起的。微脉管系统异常,如小动脉和毛细血管,通常涉及。此外,MAHA也可以通过血管内设备如人工心脏瓣膜或辅助技术来实现。不良的畸形会导致被困,吞噬作用,抗体介导的通过吞噬作用或直接补体激活的消除,由微血栓或急性机械应力引起的碎裂,氧化,或是自发的细胞死亡.溶血可能导致急性贫血,黄疸,血尿,呼吸困难,疲倦,心动过速,可能还有低血压.本文旨在综合现有研究,确定治疗策略,并提供对当前和新兴的治疗这种复杂血液病的方法的见解。
Red blood cells (RBCs) start to break down early in hemolytic anemia, which can be chronic or life-threatening. It should be considered while determining if normocytic or macrocytic anemia is present. Hemolysis in the reticuloendothelial system may happen intravascularly, extravascularly, or both. It accounts for a broad spectrum of laboratory and clinical situations, both physiological and pathological. Whenever the frequency of RBC breakdown is rapid enough to lower hemoglobin levels below the normal range, hemolytic anemia occurs. Microangiopathic hemolytic anemia (MAHA) is a term used to describe non-immune hemolysis induced by intravascular RBC fragmentation caused by substances in the tiny blood arteries that generate
schistocytes in the peripheral circulation. Microvasculature abnormalities, such as small arterioles and capillaries, are usually involved. Furthermore, MAHA can also be brought on by intravascular devices like a prosthetic heart valve or assistive technologies. Poor deformity results in entrapment, phagocytosis, antibody-mediated elimination through phagocytosis or direct complement activation, fragmentation brought about by microthrombi or acute mechanical stress, oxidation, or spontaneous cellular death. Hemolysis may cause acute anemia, jaundice, hematuria, dyspnea, tiredness, tachycardia, and possibly hypotension. This article aims to synthesize existing research, identify therapeutic strategies, and provide insights into current and emerging approaches for managing this complex hematological disorder.