title: T-cell abnormalities in common variable immunodeficiency: the hidden defect.
abstract: This review discusses how the T-cell compartment in common variable immunodeficiency is marked by the premature arrest in thymic output, leading to T-cell exhaustion and immune dysregulation. Although B cells have been the main focus of the disorder, ample experimental data suggest that T-cell abnormalities can be seen in a large proportion of Freiburg Group 1a patients and those suffering from inflammatory complications. The reductions in T-cell receptor excision circles, naïve T cells, invariant NKT cells and regulatory T cells suggest a diminished thymic output, while CD8 T cells are driven towards exhaustion either via an antigen-dependent or an antigen-independent manner. The theoretical risk of anti-T-cell therapies is discussed, highlighting the need for an international effort in generating longitudinal data in addition to better-defined underlying molecular characterisation.
authors.affiliation: MRC Centre for Immune Regulation, University of Birmingham, Birmingham, UK West Midlands Primary Immunodeficiency Centre, Birmingham Heartlands Hospital, Birmingham, UK.
authors.forename: Gabriel K
authors.initials: GK
authors.lastname: Wong
authors.affiliation: MRC Centre for Immune Regulation, University of Birmingham, Birmingham, UK West Midlands Primary Immunodeficiency Centre, Birmingham Heartlands Hospital, Birmingham, UK.
authors.forename: Aarnoud P
authors.initials: AP
authors.lastname: Huissoon
mesh_terms: D001402:B-Lymphocytes / Q000276:immunology; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology*; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology*; D017074:Common Variable Immunodeficiency / Q000276:immunology*; D006801:Humans
keywords: AUTOIMMUNITY; CMV; IMMUNODEFICIENCY; IMMUNOGLOBULIN; LYMPHOCYTES