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oai:repisalud.isciii.es:20.500.12105/204122024-11-29T06:06:01Zcom_20.500.12105_15322com_20.500.12105_2051col_20.500.12105_16967

00925njm 22002777a 4500 dc Cunill, Vanesa author Clemente, Antonio author Lanio, Nallibe author Barcelo, Carla author Andreu, Valero author Pons De Ves, Jaime author Ferrer Balaguer, Juana Maria author 2017-02-27 Germinal center follicular T helper (GCTfh) cells are essential players in the differentiation of B cells. Circulating follicular T helper (cTfh) cells share phenotypic and functional properties with GCTfh cells. Distinct subpopulations of cTfh with different helper capabilities toward B cells can be identified: cTfh1 (CXCR3(+)CCR6(-)), cTfh2 (CXCR3(-)CCR6(-)), and cTfh17 (CXCR3(-)CCR6+). Alterations in cTfh function and/or distribution have been associated with autoimmunity, infectious diseases, and more recently, with several monogenic immunodeficiencies. Common variable immunodeficiency (CVID) disease is the commonest symptomatic primary immunodeficiency with a genetic cause identified in only 2-10% of patients. Although a heterogeneous disease, most patients show a characteristic defective B cell differentiation into memory B cells or antibody-secreting cells. We investigated if alterations in CVID cTfh cells frequency or distribution into cTfh1, cTfh2, and cTfh17 subpopulations and regulatory follicular T (Tfr) cells could be related to defects in CVID B cells. We found increased percentages of cTfh exhibiting higher programmed death-1 expression and altered subpopulations distribution in smB(-) CVID patients. In contrast to smB(+) patients and controls, cTfh from smB-CVID patients show increased cTfh1 and decreased cTfh17 subpopulation percentages and increased CXCR3(+)CCR6(+) cTfh, a population analogous to the recently described pathogenic Th17.1. Moreover, Tfr cells are remarkably decreased only in smB-CVID patients. In conclusion, increased cTfh17.1 and cTfh1/cTfh17 ratio in CVID patients could influence B cell fate in smB-CVID patients, with a more compromised B cell compartment, and the decrease in Tfr cells may lead to high risk of autoimmune conditions in CVID patients. Cunill Monjo V, Clemente A, Lanio N, Barcelo C, Andreu Matillas V, Pons De Ves J, et al. Follicular T cells from smB(-) common Variable immunodeficiency Patients are skewed Toward a Th1 Phenotype. Front Immunol. 2017 Feb 27;8:174. 10.3389/fimmu.2017.00174 1664-3224 Frontiers in Immunology http://hdl.handle.net/20.500.13003/9941 28289412 L614636597 2-s2.0-85014309654 http://hdl.handle.net/20.500.12105/20412 394810200001 B cells CVID Follicular T helper cells Regulatory follicular T cells Th17.1 cells Follicular T cells from smB(-) common Variable immunodeficiency Patients are skewed Toward a Th1 Phenotype