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Table of Contents > Health Dictionary > Omenn syndrome

Omenn syndrome

A rapidly fatal immunodeficiency disease characterized by erythroderma, diarrhea, repeated infections, hepatosplenomegaly, and leukocytosis with eosinophilia; autosomal recessive inheritance, caused by mutation in either the recombination activating gene 1 (RAG1) or the adjacent RAG2 gene on chromosome 11p.

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