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IEI influencing the T-cell repertoire can manifest as severe atopy. (A) Left, resting polyclonal T cells in a normal (WT) immune repertoire. Right, oligoclonal expansion of T cells in leaky SCID, often leading to Th2 skewing and eosinophilia (Omenn syndrome). (B) Left, normal balance between regulatory T cell (Treg) and effector T cell (Teff) functions. Right, mutations affecting Treg number or function leading to autoimmune and atopic phenotypes.
