Abstract
Omenn syndrome (OS) is a rare variant of severe combined immunodeficiency characterized by autologous graft-versus-host–like disease (GVHLD) manifestations (erythroderma, hepatomegaly, diarrhea, alopecia, failure to thrive) and susceptibility to severe opportunistic infections. The typical form of the disease is caused by mutations of the recombination-activating genes (RAG1 and RAG2) that lead to an impairment of the V (D) J recombination process, the somatic diversification of T-cell receptors (TCR) and immunoglobulins.
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