Abstract
Survival of lymphocytes after prolonged culture was studied in two asymptomatic XLP patients. Viability of XLP PBMC after 30 days of non-stimulated culture was higher than that of normal controls (N), mainly due to the persistence of CD8 memory lymphocytes. IFNgamma high CD8 T lymphocytes remained higher in XLP than in N after 30 days. The number of perforin+ CD8 lymphocytes was markedly reduced after 30 days in XLP and in N. Increased viability was not related to CD127, PD-1, CD27, or CD62L expression. Concerning B lymphocytes, memory CD27+ CD19+ cells prevailed over CD27- cells after 30 days in both XLP and N, with far more surviving cells in XLP. In N, few CD19+ B lymphocytes were viable after prolonged culture. In XLP, these cells were also IgD+, IgM+ and EBNA2+. These results demonstrate that IFNgamma-positive memory CD8 T cells persist in XLP after prolonged culture in association with a subset of viable memory CD27+ B cells expressing latent EBV antigens. The survival advantage of XLP cells might be related to increased frequency of extranodal lymphoma in XLP patients.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Adult
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Antigens, CD / metabolism
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Apoptosis Regulatory Proteins / metabolism
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B-Lymphocytes / immunology
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B-Lymphocytes / pathology*
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B-Lymphocytes / virology
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CD4-Positive T-Lymphocytes / immunology
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CD4-Positive T-Lymphocytes / pathology*
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CD4-Positive T-Lymphocytes / virology
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CD8-Positive T-Lymphocytes / immunology
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CD8-Positive T-Lymphocytes / pathology*
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CD8-Positive T-Lymphocytes / virology
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Cell Survival
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Cells, Cultured
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Chromosomes, Human, X / genetics
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Epstein-Barr Virus Infections / complications
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Humans
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Interferon-gamma / metabolism
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Interleukin-7 Receptor alpha Subunit / metabolism
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Intracellular Signaling Peptides and Proteins / genetics
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Intracellular Signaling Peptides and Proteins / metabolism*
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Lymphoproliferative Disorders / immunology
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Lymphoproliferative Disorders / pathology*
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Lymphoproliferative Disorders / virology
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Perforin / metabolism
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Programmed Cell Death 1 Receptor
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Signaling Lymphocytic Activation Molecule Associated Protein
Substances
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Antigens, CD
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Apoptosis Regulatory Proteins
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Interleukin-7 Receptor alpha Subunit
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Intracellular Signaling Peptides and Proteins
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PDCD1 protein, human
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Programmed Cell Death 1 Receptor
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SH2D1A protein, human
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Signaling Lymphocytic Activation Molecule Associated Protein
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Perforin
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Interferon-gamma