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网织红细胞增多、低色素血症和小红细胞症:白血病前期综合征的一种不寻常表现。

Reticulocytosis, hypochromia, and microcytosis: an unusual presentation of the preleukemic syndrome.

作者信息

Tulliez M, Testa U, Rochant H, Henri A, Vainchenker W, Touboul J, Breton-Gorius J, Dreyfus B

出版信息

Blood. 1982 Feb;59(2):293-9.

PMID:6173084
Abstract

Two patients exhibiting a highly unusual preleukemic syndrome with marked reticulocytosis, hypochromia, and microcytosis are reported. This red cell phenotype has been investigated by means of HbF, HbA2, and i-antigen activity dosages, immunofluorescence labeling of F cells, reticulocyte survival, globin chain synthesis, and electron microscopy study. The marked reticulocytosis is explained by a delayed disappearance of the reticulum. Serum iron is normal, and a thalassemic syndrome is excluded because of a balanced alpha/non-alpha globin chain synthesis. Electron microscopy studies are consistent with a defect in iron uptake by erythroid cells. All the hematologic data and investigations are similar to those observed for the Belgrade laboratory rat. It is hypothesized that the low expression of HbF and i-Ag associated with microcytosis are related to a prolongation of erythroid maturation as reflected by abnormal reticulocyte survival.

摘要

报告了两名表现出高度异常的白血病前期综合征的患者,伴有明显的网织红细胞增多、低色素血症和小红细胞症。已通过HbF、HbA2和i抗原活性测定、F细胞的免疫荧光标记、网织红细胞存活、珠蛋白链合成以及电子显微镜研究对这种红细胞表型进行了调查。明显的网织红细胞增多是由网状结构延迟消失所解释的。血清铁正常,由于α/非α珠蛋白链合成平衡,排除了地中海贫血综合征。电子显微镜研究与红系细胞铁摄取缺陷一致。所有血液学数据和检查结果与贝尔格莱德实验大鼠所观察到的相似。据推测,与小红细胞症相关的HbF和i-Ag低表达与网织红细胞存活异常所反映的红系成熟延长有关。

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