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急性红系增生期间的胎儿血红蛋白生成。I. 对短暂性成红细胞减少症患者和放血后的观察。

Fetal Hb production during acute erythroid expansion. I. Observations in patients with transient erythroblastopenia and post-phlebotomy.

作者信息

Papayannopoulou T, Vichinsky E, Stamatoyannopoulos G

出版信息

Br J Haematol. 1980 Apr;44(4):535-46. doi: 10.1111/j.1365-2141.1980.tb08707.x.

Abstract

In order to study fetal haemoglobin production during acute erythroid expansion we did sequential measurements of Hb F-containing erythrocytes (F-cells) and of relevant haematological parameters in 10 subjects recovering from eyrthroid aplasia, iron deficiency anaemia or following phlebotomy. An increased production of F-cells was consistently observed during the acute marrow expansion, but there were significant differences in the maximum F-cell response among individuals. These differences could not be explained by differences in the degree of anaemia alone, nor could they be correlated with the level of peak reticulocytosis. Two patients who reached the highest F-cell numbers were probably carriers of heterocellular hereditary persistence of Hb F, suggesting that this gene may play a role in determining the magnitude of F-cell production in anaemic patients. It is speculated that the main reason for the consistently observed increase in F cell production during acute marrow expansion is the premature terminal differentiation of earlier erythroid precursos (burst forming units; BFUe). This proposition is in accord with observations in vitro which suggest that Hb F is expressed en eyrthroid clones derived from BFUne's. It is further proposed that differences in the degree of BFUe recruitment may underly the disparities in Hb F response among individuals subjected to similar anaemic stimuli.

摘要

为了研究急性红系增生期间胎儿血红蛋白的产生情况,我们对10名从红系再生障碍、缺铁性贫血恢复或放血后的受试者,连续测量了含Hb F的红细胞(F细胞)及相关血液学参数。在急性骨髓增生期间,始终观察到F细胞产量增加,但个体间最大F细胞反应存在显著差异。这些差异不能仅用贫血程度的差异来解释,也与网织红细胞增多峰值水平无关。两名F细胞数量达到最高的患者可能是Hb F异细胞遗传性持续存在的携带者,这表明该基因可能在决定贫血患者F细胞产生量方面发挥作用。据推测,在急性骨髓增生期间持续观察到F细胞产量增加的主要原因是早期红系前体细胞(爆式集落形成单位;BFUe)的过早终末分化。这一观点与体外观察结果一致,即Hb F在源自BFUe的红系克隆中表达。进一步提出,在受到类似贫血刺激的个体中,BFUe募集程度的差异可能是Hb F反应差异的基础。

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