A. D. B. Webster

TL;DR: Heterozygosity with respect to mutations causing the amino acid substitutions S144X and C104R abrogated APRIL binding and resulted in loss of TACI function, as evidenced by impaired proliferative response to IgM-APRIL costimulation and defective class switch recombination induced by IL-10 and APRIL or BAFF.

TL;DR: Comparison of cytokine production in cells from patients with the disease CVID showed normal cellular levels of ability to produce IL‐2 and TNF‐α but significantly raised levels of production of IFN‐γ in both CD4+ and CD8+ lymphocytes, suggesting that the pathology of this disease may involve an excessive Th1‐type response.

TL;DR: The two pathological CD28 subsets of CD8+ cells found in CVID may both be detrimental to a normal CD4‐dependent immune response and the CD28+ cytotoxic subset is over‐producing the Th1 cytokine IFN‐γ.

TL;DR: Serial studies on a small number of patients suggest that IVIG therapy has no short‐term effect on NK cells, although it cannot exclude an effect with prolonged use, and further analysis of NK cell subsets showed a deficiency of both CD16+ and CD56+ cells in CVID, which may be a factor in their predisposition to cancer.

TL;DR: An increase in TNF‐α and IL‐2 expression, combined with unchanged IFN‐γ expression, is evidence against the putative ‘anti‐inflammatory’ role of IVIG, and may explain the failure of resolution of granulomata in CVID patients treated with IVIG alone.