Grieger M, Bier K
Folia Haematol Int Mag Klin Morphol Blutforsch. 1982;109(2):290-306.
Red blood cells of 30 patients with G6PD deficiency were separated and characterized by means of isopyknic dextran density gradient centrifugation. The simultaneous determination of G6PD activity and the percentage of NADPH deficiency cells in relation to the maturation parameters of density, reticulocyte share, GOT and PK activity made it possible to recognize differences in the maturation of red blood cells with G6PD deficiency in normal persons as well as within a group of patients. In each case the more or less diminished enzyme activity of the cell suspension was accompanied by a marked enzyme deficiency of the youngest fraction. It is possible that NADPH defect cells are being eliminated at first. In many cases a direct correlation between the percentage of "empty cells" and the in vitro stability tests with and without NADP+ addition could be identified. Decreased maximal speed, changed kinetic behaviour, and instability of these variants are stressed as being the decisive parameters for the life expectation of red blood cells in patients with G6PD deficiency.
采用等密度葡聚糖密度梯度离心法对30例葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症患者的红细胞进行分离和鉴定。同时测定G6PD活性以及与密度、网织红细胞比例、谷草转氨酶(GOT)和丙酮酸激酶(PK)活性等成熟参数相关的NADPH缺乏细胞百分比,使得识别正常人和一组患者中G6PD缺乏症患者红细胞成熟的差异成为可能。在每种情况下,细胞悬液中或多或少降低的酶活性都伴随着最年轻部分明显的酶缺乏。首先可能是NADPH缺陷细胞被清除。在许多情况下,可以确定“空细胞”百分比与添加和不添加NADP+的体外稳定性试验之间存在直接相关性。强调这些变体的最大速度降低、动力学行为改变和不稳定性是G6PD缺乏症患者红细胞寿命预期的决定性参数。