title: CD4+ T Lymphocytes with follicular helper phenotype (T(FH)) in patients with SH2D1A deficiency (XLP).
abstract: Peripheral blood mononuclear cells with T(FH) phenotype from two asymptomatic XLP patients were studied. Normal/high numbers of CXCR5+, CD4+ T cells coexpressing PD-1 were demonstrated. Peripheral blood mononuclear cells (PBMC) from these patients responded to sub-optimal PHA/IL-2 stimulation upregulating ICOS and CD40L and increasing intracellular expression of IL-10, IL-21 and IL-4 by CD4+ T(FH) cells. However when compared to N, the time profile of activation and cytokine synthesis was different in XLP and N. While ICOS and CD40L expression in N decreased after 6-8 days, it continued to increase or was maintained in XLP cultures. Intracellular IL-10, IL-21 and IL-4 reached higher values in XLP than N after 8 days. Rather than the absence of T(FH) cells or their intrinsic inability to respond to stimuli, differences in the time profile of their response could contribute to impair their role as helpers of B lymphocytes.
authors: Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Buenos Aires, Argentina. anacoraglia@hematologia.anm.edu.ar
authors: Ana
authors: A
authors: Coraglia
authors: Martha
authors: M
authors: Felippo
authors: Pablo
authors: P
authors: Schierloh
authors: Alejandro
authors: A
authors: Malbran
authors: María M de E
authors: MM
authors: de Bracco
mesh_terms: D000328:Adult; D015496:CD4-Positive T-Lymphocytes / Q000187:drug effects / Q000276:immunology*; D023201:CD40 Ligand / Q000096:biosynthesis / Q000276:immunology; D002478:Cells, Cultured; D005091:Exons; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D060889:Inducible T-Cell Co-Stimulator Protein / Q000096:biosynthesis / Q000276:immunology; D007376:Interleukin-2 / Q000276:immunology / Q000494:pharmacology; D007378:Interleukins / Q000276:immunology / Q000378:metabolism; D047908:Intracellular Signaling Peptides and Proteins / Q000235:genetics / Q000276:immunology*; D007963:Leukocytes, Mononuclear / Q000276:immunology; D008232:Lymphoproliferative Disorders / Q000188:drug therapy / Q000235:genetics / Q000276:immunology*; D008875:Middle Aged; D009154:Mutation; D010835:Phytohemagglutinins / Q000276:immunology / Q000494:pharmacology; D000071179:Signaling Lymphocytic Activation Molecule Associated Protein; D006377:T-Lymphocytes, Helper-Inducer / Q000276:immunology; D015854:Up-Regulation / Q000276:immunology
chemical_list: C556692:ICOS protein, human; D016756:Immunoglobulins, Intravenous; D060889:Inducible T-Cell Co-Stimulator Protein; D007376:Interleukin-2; D007378:Interleukins; D047908:Intracellular Signaling Peptides and Proteins; D010835:Phytohemagglutinins; C114953:SH2D1A protein, human; D000071179:Signaling Lymphocytic Activation Molecule Associated Protein; D023201:CD40 Ligand