Erythroid progenitors in the peripheral blood of children with sickle cell disease.

T B West, K Ohene-Frempong, C J Stoeckert, S Surrey
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Abstract

Purpose: The goals of this study were (a) to determine the number of peripheral blood burst forming units-erythroid (BFU-E); (b) to define the relationship between circulating BFU-E number and fetal hemoglobin (HbF) level; and (c) to define the relationship between BFU-E number and age in pediatric sickle cell disease (SCD) patients.

Patients and methods: Fetal hemoglobin (HbF) level and peripheral blood BFU-E number were determined in children < 18 years of age with SCD in a steady state of their disease. These data were compared with those of normal children.

Results: An increased number of BFU-E was observed in the peripheral blood of children with SCD compared with normals (30.7 vs. 15.7 per 10(5) mononuclear cells, respectively; p = 0.009). Overall there was the suggestion of a direct relationship between HbF level and peripheral blood BFU-E number (regression coefficient = 0.445; p = 0.06). Additionally, a strong inverse relationship between BFU-E number and age (regression coefficient = -0.671; p < 0.0001) was observed.

Conclusions: In children with SCD (a) there are an increased number of peripheral blood BFU-E compared with normal children; (b) the inverse relationship between HbF level and BFU-E number observed in adult SCD patients is not seen in children; and (c) there is a strong inverse relationship between age and BFU-E number. This information may help to further clarify the relationship between peripheral blood BFU-E and erythropoietic stress.

镰状细胞病患儿外周血中的红系祖细胞。
目的:本研究的目的是:(a)测定外周血破裂形成单位-红细胞(BFU-E)的数量;(b)确定循环BFU-E数与胎儿血红蛋白(HbF)水平的关系;(c)确定小儿镰状细胞病(SCD)患者BFU-E数与年龄的关系。患者和方法:对年龄< 18岁、病情稳定的SCD患儿进行胎儿血红蛋白(HbF)水平和外周血BFU-E数测定。这些数据与正常儿童的数据进行了比较。结果:与正常人相比,SCD患儿外周血中BFU-E的数量增加(30.7比15.7 / 10(5)单个核细胞;P = 0.009)。总体而言,HbF水平与外周血BFU-E数有直接关系(回归系数= 0.445;P = 0.06)。BFU-E值与年龄呈显著负相关(回归系数= -0.671;P < 0.0001)。结论:与正常儿童相比,SCD儿童(a)外周血BFU-E数量增加;(b)在成人SCD患者中观察到的HbF水平和BFU-E数之间的负相关关系在儿童中未见;(c)年龄与BFU-E值呈较强的负相关。这一信息可能有助于进一步阐明外周血BFU-E与红细胞生成应激之间的关系。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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