Skewed distribution of circulating activated natural killer T (NKT) cells in patients with common variable immunodeficiency disorders (CVID)

• Published in: PloS one Vol. 5; no. 9; p. e12652
• Main Authors: Carvalho, Karina I, Melo, Karina M, Bruno, Fernanda R, Snyder-Cappione, Jennifer E, Nixon, Douglas F, Costa-Carvalho, Beatriz T, Kallas, Esper G
• Format: Journal Article
• Language: English
• Published: United States Public Library of Science 09-09-2010; Public Library of Science (PLoS)
• Subjects: Adult; Adults; Autoimmune diseases; Bacteria; Case-Control Studies; CCR5 protein; CD4 antigen; CD4 Lymphocyte Count; CD69 antigen; CD8 antigen; Chemokines; Children; Cloning; Common variable immunodeficiency; Common Variable Immunodeficiency - genetics; Common Variable Immunodeficiency - immunology; Effector cells; Female; Flow cytometry; Gene Expression; Humans; Immunoglobulins; Immunology; Immunology/Autoimmunity; Immunology/Immunity to Infections; Immunology/Innate Immunity; Lymphocyte Activation; Lymphocytes; Lymphocytes T; Male; Natural killer cells; Natural Killer T-Cells - cytology; Natural Killer T-Cells - immunology; NK Cell Lectin-Like Receptor Subfamily B - genetics; NK Cell Lectin-Like Receptor Subfamily B - immunology; Patients; Penicillin; Receptors, CCR5 - genetics; Receptors, CCR5 - immunology; Skewed distributions; T cell receptors; T-Lymphocyte Subsets - cytology; T-Lymphocyte Subsets - immunology; Tuberculosis; Young Adult; Brazil; San Francisco California; California
• ISSN: 1932-6203; 1932-6203
• DOI: 10.1371/journal.pone.0012652

Common variable immunodeficiency disorder (CVID) is the commonest cause of primary antibody failure in adults and children, and characterized clinically by recurrent bacterial infections and autoimmune manifestations. Several innate immune defects have been described in CVID, but no study has yet investigated the frequency, phenotype or function of the key regulatory cell population, natural killer T (NKT) cells. We measured the frequencies and subsets of NKT cells in patients with CVID and compared these to healthy controls. Our results show a skewing of NKT cell subsets, with CD4+ NKT cells at higher frequencies, and CD8+ NKT cells at lower frequencies. However, these cells were highly activated and expression CD161. The NKT cells had a higher expression of CCR5 and concomitantly expression of CCR5+CD69+CXCR6 suggesting a compensation of the remaining population of NKT cells for rapid effector action.