Definition:
Omenn syndrome is a severe combined immunodeficiency associated with mutations in the recombination activating genes, affecting both B-cells and T-cells.
Treatment:
Patients sometimes undergo a bone marrow transplantation and cord blood stem cells.
Symptoms and Signs:
Patients may experience shedding of the skin’s outer layer, reddening of the skin, recurrent diarrhea, persistent bacterial infections, elevated count of white blood cells, enlarged liver and spleen, and swelling of lymph nodes.
Causes:
Omenn syndrome is genetically inherited.
