Irreversibly sickled erythrocytes in sickle cell anemia: A quantitative reappraisal
Irreversibly sickled erythrocytes in sickle cell anemia: A quantitative reappraisal
复制标题
镰状细胞性贫血中不可逆的镰状红细胞：定量重新评估
DOI：
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发表时间：
1985
期刊：
影响因子：
--
通讯作者：
A. Schechter
中科院分区：
文献类型：
--
作者：
G. Rodgers;C. Noguchi;A. Schechter
据报道，不可逆镰状细胞 (ISC) 被一些人认为具有重要的病理生理学意义，占纯合镰状细胞贫血患者红细胞总数的 5-50%。由于含有镰状血红蛋白的红细胞的变形高度依赖于脱氧构象中血红蛋白的浓度，因此任何为计算真实ISC计数而建立的方法都需要血红蛋白处于全氧构象或配体构象。由于镰状红细胞的氧解离曲线显着向右移动，因此需要极高的氧分压才能接近完全饱和。另一方面，可以很容易地实现氧构象中的血红蛋白与一氧化碳（CO）的完全配位。我们发现，与 ISC 制剂的传统方法相比，在 CO 中孵育后，镰状细胞性贫血患者外周血中镰状细胞的平均数量显着减少（降至 6.5 ± 3.5%）。这些结果表明，在该疾病的病理生理学研究中，应使用完全配体的红细胞来定量 ISC，特别是因为 ISC 可能与可逆镰状细胞不同地影响流变学。
Irreversibly sickled cells (ISCs), considered by some to be of major pathophysiologic significance, have been reported to comprise between 5–50% of the total red cell population in patients with homozygous sickle cell anemia. Since the deformation of erythrocytes containing sickle hemoglobin is highly dependent on the concentration of hemoglobin in the deoxy conformation, any method established to enumerate the true ISC count requires the hemoglobin to be in the full oxy or liganded conformation. Because the oxygen dissociation curve for sickle erythrocytes is significantly shifted to the right, extremely high partial pressures of oxygen are required to approach full saturation. On the other hand, fully liganding the hemoglobin in the oxy conformation with carbon monoxide (CO) can be readily accomplished. We found that there is a significant reduction in the average number of sickled forms in the peripheral blood of sickle cell anemia patients after incubation in CO (to a value of 6.5 ± 3.5%) when compared to conventional methods for ISC preparations. These results suggest that fully liganded erythrocytes should be used in quantitating ISCs in studies of the pathophysiology of this disease, especially since ISCs are likely to affect rheology differently from reversibly sickling cells.
体外二氧化碳和 pH 变化对血液呼吸功能、红细胞体积、跨膜 pH 梯度和镰状细胞性贫血中镰状化的影响。
DOI：
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发表时间：
1984
期刊：
The Journal of laboratory and clinical medicine
影响因子：
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作者：
Ueda,Y;Bookchin,RM
通讯作者：
Bookchin,RM
镰状红细胞的吞噬作用：溶血性贫血的免疫和氧化决定因素。
影响因子：
20.3
作者：
Hebbel,RP;Miller,WJ
通讯作者：
Miller,WJ