Omenn syndrome
Omenn syndrome is a rare immunodeficiency disease. It is an autosomal recessive form of severe combined immunodeficiency (SCID).
Symptoms
Symptoms include:
- Desquamation (shedding the outer layers of skin)
- Chronic diarrhea
- Erythroderma (widespread reddening of the skin)
- Hepatosplenomegaly (simultaneous enlargement of both the liver and the spleen)
- Leukocytosis (elevated of the white blood cell count)
- Lymphadenopathy (swelling of one or more lymph nodes)
- Repeated bacterial infections
Treatment
Omenn syndrome is amongst the diseases treated with bone marrow transplantation and cord blood stem cells.