T-cell abnormalities in common variable immunodeficiency: the hidden defect
GK Wong, AP Huissoon - Journal of clinical pathology, 2016 - jcp.bmj.com
Journal of clinical pathology, 2016•jcp.bmj.com
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 …
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 …
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.
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