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.
Copyright © 2011 Elsevier Inc. All rights reserved.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Adult
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CD4-Positive T-Lymphocytes / drug effects
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CD4-Positive T-Lymphocytes / immunology*
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CD40 Ligand / biosynthesis
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CD40 Ligand / immunology
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Cells, Cultured
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Exons
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Humans
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Immunoglobulins, Intravenous / therapeutic use
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Inducible T-Cell Co-Stimulator Protein / biosynthesis
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Inducible T-Cell Co-Stimulator Protein / immunology
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Interleukin-2 / immunology
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Interleukin-2 / pharmacology
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Interleukins / immunology
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Interleukins / metabolism
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Intracellular Signaling Peptides and Proteins / genetics
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Intracellular Signaling Peptides and Proteins / immunology*
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Leukocytes, Mononuclear / immunology
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Lymphoproliferative Disorders / drug therapy
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Lymphoproliferative Disorders / genetics
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Lymphoproliferative Disorders / immunology*
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Middle Aged
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Mutation
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Phytohemagglutinins / immunology
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Phytohemagglutinins / pharmacology
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Signaling Lymphocytic Activation Molecule Associated Protein
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T-Lymphocytes, Helper-Inducer / immunology
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Up-Regulation / immunology
Substances
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ICOS protein, human
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Immunoglobulins, Intravenous
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Inducible T-Cell Co-Stimulator Protein
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Interleukin-2
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Interleukins
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Intracellular Signaling Peptides and Proteins
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Phytohemagglutinins
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SH2D1A protein, human
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Signaling Lymphocytic Activation Molecule Associated Protein
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CD40 Ligand