Coulombel L, Tchernia G, Mielot F, Mohandas N
Exp Hematol. 1984 Aug;12(7):587-93.
Defective hemoglobin synthesis and gross morphologic abnormalities of erythroblasts are striking features of hematopoietic dysplasia. In order to define the effects of defective hemoglobin synthesis on hemoglobin content of erythroblasts at each stage of maturation, we measured the hemoglobin content and concentration of individual erythroblasts at various stages of maturation in the bone marrow by scanning microspectrophotometry. In contrast to normal erythroblasts, those from hematopoietic dysplasia had decreased hemoglobin content and decreased hemoglobin concentration at late stages of maturation. This observation, together with the observed near-normal hemoglobin content and concentration of circulating red cells in this disorder, suggests that polychromatophilic erythroblasts with decreased hemoglobin content fail to complete normal maturation. The abnormalities in hemoglobin content observed in vivo in hematopoietic dysplasia could be reproduced in the in vitro cultures of erythroid progenitors of bone marrow cells from the patients.
血红蛋白合成缺陷和幼红细胞的明显形态学异常是造血发育异常的显著特征。为了确定血红蛋白合成缺陷对各成熟阶段幼红细胞血红蛋白含量的影响，我们通过扫描显微分光光度法测量了骨髓中不同成熟阶段单个幼红细胞的血红蛋白含量和浓度。与正常幼红细胞相比，造血发育异常患者的幼红细胞在成熟后期血红蛋白含量降低，血红蛋白浓度也降低。这一观察结果，连同该疾病中循环红细胞血红蛋白含量和浓度接近正常的现象，表明血红蛋白含量降低的嗜多色性幼红细胞无法完成正常成熟。造血发育异常患者体内观察到的血红蛋白含量异常在来自患者的骨髓细胞红系祖细胞的体外培养中能够重现。
