T-cell abnormalities in common variable immunodeficiency: the hidden defect

Gabriel K Wong, Aarnoud P Huissoon

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21 Citations (Scopus)
150 Downloads (Pure)


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.
Original languageEnglish
Pages (from-to)672-676
JournalJournal of Clinical Pathology
Issue number8
Early online date6 May 2016
Publication statusPublished - Aug 2016


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