AI Article Synopsis
- The study investigates how thymic dysplasia affects T cells in patients with 22q11.2 deletion syndrome, focusing on their characteristics and response to IL-7 treatment.
- Findings show a strong Th1 response in these patients, characterized by specific T cell expansions and increased production of various cytokines like IFN-γ and IL-10.
- IL-7 treatment leads to a reduction in naive T cells and an increase in exhaustion markers, indicating that the Th1 bias remains through adolescence and promotes early maturation of T cells.
Article Abstract
The aim of this study was to investigate the impact of thymic dysplasia on the phenotypic and functional characteristics of T cells in patients with 22q11.2 deletion syndrome, including T-cell phenotype, transcriptional profile, cytokine production, as well as the possibility of utilizing IL-7 to recover their numbers and function. We found a strong bias towards Th1 response in pediatric and young adult 22q11.2DS patients, expansion of CXCR5 follicular helper cells and CXCR3CCR6 Th1 cells, increased production of cytokines IFN-γ, IL-10, IL-2, IL-21 and TNF-α. This Th1 skew was primarily driven by expanded terminally differentiated T cells. IL-7 further reduced naive T cells, increased cytokine production and caused an upregulation of exhaustion markers. Thus, Th1 bias in T cell populations persists from infancy into adolescence and is accompanied by accelerated maturation of T cells into memory stages. This phenotype is exacerbated by IL-7 which causes further decrease in naïve T cells in vitro.
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| http://dx.doi.org/10.1016/j.clim.2023.109793 | DOI Listing |
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