Oligoclonality of the TCR repertoire was previously demonstrated; however, the manner in which it relates to other B cell and T cell findings reported in CVID remains unclear.
Our results demonstrate that the frequency of CD1d-restricted TCR invariant natural killer T cells in PB was significantly reduced in the patients with CVID.
These data indicate that the patient with CVID has a defect in TCR-mediated signalling at the level of the T cells which is not present in his histoidentical healthy brothers or in his haploidentical IgA-deficient sister.