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抗体缺陷儿童中的CXCR5 T滤泡辅助细胞区室——寻找儿童低丙种球蛋白血症的预后标志物

The CXCR5 T follicular helper cell compartment in children with antibody deficiencies-in search of a prognostic marker of childhood hypogammaglobulinemia.

作者信息

Szczawinska-Poplonyk Aleksandra, Tapolska-Jozwiak Katarzyna, Samara Husam, Boruczkowski Maciej, Wieckowska Barbara

机构信息

Department of Pediatric Pneumonology, Allergology and Clinical Immunology, Poznan University of Medical Sciences, Poznan, Poland;

Department of Pediatric Pneumonology, Allergology and Clinical Immunology, Poznan University of Medical Sciences, Poznan, Poland.

出版信息

Allergol Immunopathol (Madr). 2021 Mar 1;49(2):113-121. doi: 10.15586/aei.v49i2.34. eCollection 2021.

DOI:10.15586/aei.v49i2.34
PMID:33641302
Abstract

BACKGROUND

Novel immunodiagnostic markers are required in order to discriminate between mild hypogammaglobulinemia and severe humoral primary immune deficiencies in children. The efficacy of an antibody response to infections and vaccines is underpinned by T follicular helper (Tfh) cells, activating an immunoglobulin class switch recombination, somatic hypermutations, and affinity maturation.

OBJECTIVE

To determine the formation of the Tfh cells in antibody deficient children and to define their importance as prognostic markers helpful in defining the severity of hypogammaglobulinemia.

METHODS

We retrospectively reviewed medical records of 200 children aged from 2 months to 10 years, in whom hypogammaglobulinemia was assessed, from January to December 2019. In all the children studied, a flow cytometric analysis of the Tfh cell compartment was performed.

RESULTS

In young infants aged from 2 to 9 months, the mean relative frequency of the Tfh population was lower than in the control population. Concomitantly, the relative values of Tfh cells, corresponding with the 95th percentile, were below the reference values in all age groups.

CONCLUSIONS

A deficiency of Tfh cells in young infants mirrors the immaturity of the humoral immune response, whereas in older children Tfh cells are proposed as a prognostic marker facilitating to distinguish between mild hypogammaglobulinemia and the developing common variable immunodeficiency.

摘要

背景

为了区分儿童轻度低丙种球蛋白血症和严重体液原发性免疫缺陷,需要新型免疫诊断标志物。对感染和疫苗的抗体反应的效力由滤泡辅助性T(Tfh)细胞支持,其可激活免疫球蛋白类别转换重组、体细胞超突变和亲和力成熟。

目的

确定抗体缺陷儿童中Tfh细胞的形成,并确定其作为有助于定义低丙种球蛋白血症严重程度的预后标志物的重要性。

方法

我们回顾性分析了2019年1月至12月评估有低丙种球蛋白血症的200名2个月至10岁儿童的病历。在所有研究儿童中,均进行了Tfh细胞亚群的流式细胞术分析。

结果

在2至9个月的幼儿中,Tfh细胞群的平均相对频率低于对照组。同时,所有年龄组中对应第95百分位数的Tfh细胞相对值均低于参考值。

结论

幼儿中Tfh细胞的缺乏反映了体液免疫反应的不成熟,而在大龄儿童中,Tfh细胞被认为是一种预后标志物,有助于区分轻度低丙种球蛋白血症和正在发展的常见可变免疫缺陷。

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引用本文的文献

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Immune Dysregulation in Pediatric Common Variable Immunodeficiency: Implications for the Diagnostic Approach.儿童常见变异型免疫缺陷中的免疫失调:对诊断方法的启示
Front Pediatr. 2022 Mar 23;10:855200. doi: 10.3389/fped.2022.855200. eCollection 2022.