Omenn syndrome
From Wikipedia, the free encyclopedia
| ICD-10 | D81.2 (ILDS D81.210) |
|---|---|
| OMIM | 603554 |
| DiseasesDB | 32676 |
| eMedicine | ped/1640 |
Omenn syndrome is a rare immunodeficiency disease. It is an autosomal recessive form of severe combined immunodeficiency (SCID).
[edit] 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
[edit] Treatment
Omenn syndrome is among the diseases treated with bone marrow transplantation and cord blood stem cells.
