Ohba Y
Department of Clinical Laboratory Science, Yamaguchi University School of Medicine.
Nihon Rinsho. 1996 Sep;54(9):2376-81.
The process of denaturation of Hb within red cells, leading to ultimate formation of Heinz body or aggregates of irreversibly denatured Hb, was formulated over 20 years ago. The mechanical obstruction due to Heinz bodies seems self-evident as a cause of enhanced red cell destruction. Recent studies compare the overall defects in rheologic properties of red cells of unstable Hb disorder with artificially oxidized red cells. The fate of superoxide anion which is the other product of oxidative denaturation of Hb is being discussed in the light of accumulation of oxidative damage to red cell membrane. From this aspect, unstable Hb disorder may be regarded as a model of accelerated red cell aging. The aged red cells appear to bind increased amounts of immunoglobulin. They may express scavenger ligands. The rheologic defects and altered surface phenomena would be additive in enhancing red cell phagocytosis. The chemical basis for the abnormal properties of unstable Hb red cells, as well as normal senescent red cells, is yet to be defined.
红细胞内血红蛋白变性的过程，最终导致亨氏小体或不可逆变性血红蛋白聚集体的形成，这一过程早在20多年前就已被阐述。亨氏小体造成的机械性阻塞作为红细胞破坏增加的一个原因似乎是不言而喻的。最近的研究将不稳定血红蛋白病红细胞的流变学特性的总体缺陷与人工氧化的红细胞进行了比较。鉴于红细胞膜氧化损伤的积累，正在讨论血红蛋白氧化变性的另一种产物超氧阴离子的命运。从这个角度来看，不稳定血红蛋白病可被视为红细胞加速老化的一个模型。老化的红细胞似乎结合了更多的免疫球蛋白。它们可能表达清除剂配体。流变学缺陷和改变的表面现象在增强红细胞吞噬作用方面会起到累加作用。不稳定血红蛋白红细胞以及正常衰老红细胞异常特性的化学基础尚未明确。
