Noguchi C T, Schechter A N
Am J Pediatr Hematol Oncol. 1984 Spring;6(1):46-50.
Intact sickle erythrocytes were fractionated to obtain a more uniform population in mean corpuscular hemoglobin concentration (MCHC). Polymer fraction at complete deoxygenation varied with increasing MCHC and, at varying oxygen saturation, was in good agreement with theoretical predictions based on solubility and the non-ideal behavior of concentrated hemoglobin solution and of water. These data and the theoretical analysis indicate that, in uniform sickle erythrocytes at 34 g/dl, polymer is only detected below 84% oxygen saturation. Non-uniformity of unfractionated sickle erythrocytes causes this critical oxygen saturation to shift to higher values (over 90%). The implication is that polymer may exist in arterial blood, a possibility which must be considered in therapeutic strategies. Recently, we showed that polymer formation correlated with the severity of hemolysis for 12 sickle syndromes varying from sickle trait to hemoglobin SS (African) disease.
对完整的镰状红细胞进行分级分离,以获得平均红细胞血红蛋白浓度(MCHC)更均匀的群体。完全脱氧时的聚合物级分随MCHC的增加而变化,并且在不同的氧饱和度下,与基于溶解度以及浓缩血红蛋白溶液和水的非理想行为的理论预测高度一致。这些数据和理论分析表明,在34 g/dl的均匀镰状红细胞中,仅在氧饱和度低于84%时才能检测到聚合物。未分级的镰状红细胞的不均匀性导致这个临界氧饱和度向更高值(超过90%)移动。这意味着聚合物可能存在于动脉血中,这是治疗策略中必须考虑的一种可能性。最近,我们表明,对于从镰状性状到血红蛋白SS(非洲人)病的12种镰状综合征,聚合物形成与溶血严重程度相关。