XLA PATIENTS DISPLAY REDUCED EXPANSION FOR BCG CELLULAR IMMUNE RESPONSE AND A LOWER IFN-Gamma PRODUCTION

Abstract

The aim of this study was to investigate whether X- Linked Agammaglobulinaemia (XLA) patients with BCG scars can alter the subset composition, activation or function of specific BCG T cells and pro and anti inflammatory cytokines. After, we investigated Mycobacterium bovis bacilli Calmette-Guerin (BCG) vaccination, administered by the intradermic route, the cellular immune response in 14 patients with XLA and compared this to 18 healthy males paired by gender and age. We examined the production of proinflammatory Th1 (IFN-g) and Th2 (IL-4), TNF-a and anti-inflammatory IL-10 cytokines in supernatant of peripheral mononuclear cell cultures in response to live BCG, and PHA to determine specific and non-specific adaptive immune responses. We observed a reduction of the proliferative response to BCG and PHA, as well as a reduction in IFN- γ production in response to BCG. The proportion of blast CD4, CD8 and γδ T cells is preserved (data not shown), indicating that the lymphocyte subpopulations are usually represented, but that the proliferative response is decreased. Furthermore, in vitro T cell activation, in response to live BCG and PHA signals, leads to a lower IFN-g production although the cell culture spontaneously produces normal TNF-a and IL-10. These data agree with results recently described that effector and regulatory B cell subsets modulate the function of T cells.