Increased lymphocyte viability after non-stimulated peripheral blood mononuclear cell (PBMC) culture in patients with X-linked lymphoproliferative disease (XLP).
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.
Belmonte
Liliana
L
Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, 1425 Buenos Aires, Argentina.
Parodi
Cecilia
C
Bastón
Mariela
M
Coraglia
Ana
A
Felippo
Marta
M
Baré
Patricia
P
Malbrán
Alejandro
A
Ruibal-Ares
Beatriz
B
de E de Bracco
María M
MM
D000328:Adult; D015703:Antigens, CD / Q000378:metabolism; D051017:Apoptosis Regulatory Proteins / Q000378:metabolism; D001402:B-Lymphocytes / Q000276:immunology / Q000473:pathology* / Q000821:virology; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology / Q000473:pathology* / Q000821:virology; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology / Q000473:pathology* / Q000821:virology; D002470:Cell Survival; D002478:Cells, Cultured; D041321:Chromosomes, Human, X / Q000235:genetics; D020031:Epstein-Barr Virus Infections / Q000150:complications; D006801:Humans; D007371:Interferon-gamma / Q000378:metabolism; D053699:Interleukin-7 Receptor alpha Subunit / Q000378:metabolism; D047908:Intracellular Signaling Peptides and Proteins / Q000235:genetics / Q000378:metabolism*; D008232:Lymphoproliferative Disorders / Q000276:immunology / Q000473:pathology* / Q000821:virology; D054353:Perforin / Q000378:metabolism; D061026:Programmed Cell Death 1 Receptor; D000071179:Signaling Lymphocytic Activation Molecule Associated Protein
D015703:Antigens, CD; D051017:Apoptosis Regulatory Proteins; D053699:Interleukin-7 Receptor alpha Subunit; D047908:Intracellular Signaling Peptides and Proteins; C105992:PDCD1 protein, human; D061026:Programmed Cell Death 1 Receptor; C114953:SH2D1A protein, human; D000071179:Signaling Lymphocytic Activation Molecule Associated Protein; D054353:Perforin; D007371:Interferon-gamma