Schröter W, Ungefehr K, Tillmann W
Klin Wochenschr. 1981 Feb 16;59(4):173-9. doi: 10.1007/BF01477477.
A girl of German origin with severe congenital hemolytic anemia was found to have overhydrated erythrocytes with markedly increased sodium and reduced potassium concentrations. The cells were stomatocytic with increased osmotic fragility and autohemolysis. Their excessively increased energy production was mainly used for active cation transport. The cells were highly vulnerable to metabolic depletion. Their normal deformability was reduced rapidly during deprivation of glucose. In acidic, hypoglycemic environment of the splenic pulp the cells become swollen and lysed. After splenectomy anemia improved though increased hemolysis persisted.
一名患有严重先天性溶血性贫血的德国裔女孩，其红细胞存在水合过度现象，钠浓度显著升高而钾浓度降低。细胞呈口形，渗透脆性增加且自溶血现象明显。其能量产生过度增加主要用于主动阳离子转运。这些细胞极易受到代谢耗竭的影响。在缺乏葡萄糖的情况下，它们正常的变形能力会迅速降低。在脾髓的酸性、低血糖环境中，细胞会肿胀并裂解。脾切除术后贫血有所改善，不过溶血增加的情况仍持续存在。
