Hypogammaglobulinemia in children: a warning sign to look deeply?

被引：1

作者：

de Melo, Karina Mescouto ^{[1
]}

de Moraes-Pinto, Maria Isabel ^{[1
]}

Andrade, Luis E. C. ^{[2
]}

Salomao, Reinaldo ^{[2
]}

Brunialti, Milena K. C. ^{[2
]}

Ferreira, Vanessa S. ^{[2
]}

Costa-Carvalho, Beatriz T. ^{[1
]}

机构：

[1] Univ Fed Sao Paulo, UNIFESP, Escola Paulista Med, Dept Pediat, Sao Paulo, Brazil

[2] Univ Fed Sao Paulo, UNIFESP, Escola Paulista Med, Dept Med, Sao Paulo, Brazil

来源：

APMIS | 2017年 / 125卷 / 10期

基金：

巴西圣保罗研究基金会;

关键词：

Hypogammaglobulinemia; cytokine; lymphocytes; primary immunodeficiency; children; COMMON VARIABLE IMMUNODEFICIENCY; MEMORY B-CELLS; DEFICIENCY; SUBGROUPS; SUBSETS;

D O I：

10.1111/apm.12738

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

This study investigated phenotypic and functional characteristics of lymphocytes in children with common variable immunodeficiency (CVID) and unclassified hypogammaglobulinemia (UH), as well as B-cell subsets in non-consanguineous parents. Blood samples of 30 children, CVID (n = 9), UH (n = 9), healthy donors HD (n = 12), and 19 adults (parents and controls) were labeled by a combination of surface markers to identify CD4, CD8 T-cell and B-cell sub-populations. T-cell cytokine production in children was analyzed in vitro after stimulation with phytohemagglutinin (PHA) and tetanus toxoid. We observed low percentages of switched memory B cells in children with CVID, increase in total CD4(+) T-cell counts, and high percentages of transitional B cells only in UH group. Analysis of T-cell immunity showed that CVID children had decreased percentages of CD8(+) IFN-gamma-producing cells after stimulation with PHA and tetanus toxoid. Parent of children with CVID had low percentages of naive B cell and increased percentages of memory B cells in comparison with controls. These results suggest that (i) early combined immune defect in children with CVID and (ii) a possible familial B-cell disturbance in pediatric CVID.

引用

页码：902 / 909

页数：8

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