Omenn syndrome represents which of the following immunological condition?
Omenn syndrome consists of a subset of T cell deficiencies that present with early-onset erythroderma, alopecia, hepatosplenomegaly, and failure to thrive. This peculiar syndrome is due to the hypomorphic mutations in genes usually associated with SCID, i.e., RAG-1, RAG-2,. The patients are very fragile, requiring simultaneous anti-infective therapy, nutritional suppo, and immunosuppression. Ref: Harrisons Principles of Internal Medicine, 18th Edition, Page 2701.
Human Leukocyte antigen (HLA) Class II Deficiency
HLA Class I Deficiency
Purine Metabolism Deficiency
Subset of T cell deficiency