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Significant changes in the composition of the precursor B-cell compartment in children less than 2 years old

机译:2岁以下儿童前体B细胞区隔的组成发生重大变化

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Defects in early B lymphocyte maturation in bone marrow (BM) compose a characteristic feature of many primary immune deficiencies associated with agammaglobulinemia. To date, only limited data on the composition of the precursor B-cell compartment in BM is available. The aim of this study was to define normal age-related ranges of total B-cell content and distribution of precursor B - cell stages in BM for the future use in clinical diagnostics. Methods: Four color flow cytometry was used to analyze the composition of the B-cell compartment in specimens from 59 hematologically healthy children, aged 14 days to 16 years, assigned to six age groups: neonates less than 1 month old, infants >1-12 months old, children >1-2 years old, >2-5 years old, >5-10 years old, and older than 10 years. Results: Analysis of the composition of the B-cell compartment revealed significant age-related variation in the distribution of individual B-cell maturation stages, most seriously affecting children during first 2 years of life, with the shift from domination of the earliest stages, to gradually increasing content of mature B-cells. Significantly higher proportions of pro-B lymphocytes were observed in neonates than in any other age group. Conclusion: Physiological age-related variation in the precursor B-cell compartment composition affects most seriously very young children below the age of 2 years. Proper interpretation of immunophenotyping results performed in cases of suspected early B-cell differentiation defect requires application of adequate reference data.
机译:骨髓(BM)早期B淋巴细胞成熟的缺陷是许多与丙种球蛋白血症相关的原发性免疫缺陷的特征。迄今为止,仅可获得关于BM中前体B细胞区室组成的有限数据。这项研究的目的是确定总B细胞含量和BM中前体B细胞阶段分布的正常年龄相关范围,以供将来在临床诊断中使用。方法:采用四色流式细胞术分析了59例年龄在14天至16岁之间的血液学健康儿童的B细胞区室组成,这些儿童分为六个年龄组:新生儿小于1个月,婴儿> 1- 12个月大,儿童> 1-2岁,> 2-5岁,> 5-10岁,且年龄大于10岁。结果:对B细胞区隔的组成进行分析后发现,各个B细胞成熟阶段的分布存在明显的年龄相关变化,这对生命的最初2年影响最为严重,从最早阶段的控制开始转变,逐渐增加成熟B细胞的含量。新生儿中观察到的pro-B淋巴细胞比例明显高于其他任何年龄组。结论:前体B细胞区室组成中与年龄相关的生理变化最严重地影响了2岁以下的非常年幼的儿童。在怀疑早期B细胞分化缺陷的情况下,对免疫表型结果的正确解释需要应用足够的参考数据。

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