Hypogammaglobulinemia in children: a warning sign to look deeply?

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.Sao Paulo Research Foundation (FAPESP)Coordenacao de Aperfeicoamento de Pessoal de Nivel Superior (CAPES), Brazilian Ministry of EducationUniv Fed Sao Paulo, UNIFESP, Escola Paulista Med, Dept Pediat, Sao Paulo, BrazilUniv Fed Sao Paulo, UNIFESP, Escola Paulista Med, Dept Med, Sao Paulo, BrazilUniv Fed Sao Paulo, UNIFESP, Escola Paulista Med, Dept Pediat, Sao Paulo, BrazilUniv Fed Sao Paulo, UNIFESP, Escola Paulista Med, Dept Med, Sao Paulo, BrazilFAPESP: 2010/20834-5Web of Scienc