Peripheral blood follicular T cells (CTFH) in patients with primary immunodeficiencies

CALDIROLA, MARÍA SOLEDAD; SEMINARIO, ANALÍA GISELA; ZWIRNER, NORBERTO WALTER; GAILLARD, MARÍA ISABEL; BEZRODNIK, LILIANA

Buenos Aires

Congreso; Reunión Conjunta de Sociedades de Biociencias y 64a Reunión Anual de la Sociedad Argentina de Inmunología; 2017

Sociedad Argentina de Inmunología

Introduction: T follicular helper (Tfh) cells represent a CD4+ T cell subset specialized to provide help to B cells and to induce durable antibody response. Tfh cells are essential for the formation of germinal centers (GCs). Peripheral blood CXCR5+ CD4+ T cells are currently recognized as a circulating counterpart of Tfh cells (cTfh) and are a heterogeneous group composed of distinct subsets with a characteristic phenotype and function. AIM: describe cTfh cells in peripheral blood of CD4+memory cells from healthy donors (n=14) and patients with PIDs due to GC defect (2 BTK mutation, 1 CD40L mutation, 1 STAT-3 LOF mutation) and patients with dysregulatory syndromes (1 CD25 mutation, 2 STAT-1 GOF mutation, 1 STAT-5b mutation, 2 IDCV patients) were stained with anti-human-CXCR5, CCR6 and CXCR3 by flow cytometry (FC). RESULTS: HD had a cTfh percentage mean of 12,7 ± 3,6 (X±SD). Four subsets of cTfh cells were defined: cTfh1 (CXCR3+), cTfh17 (CCR6+), cTfh1/17(CXCR3+ CCR6+), and cTfh2 (CXCR3-CCR6-). Patients with PIDs affecting GC development showed a quantitative reduction in cTfh (12,7 vs 4,8) (p