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Omenn syndrome



Omenn syndrome
Classification & external resources
ICD-10 D81.2 (ILDS D81.210)
OMIM 603554
DiseasesDB 32676
eMedicine ped/1640 

Omenn syndrome is a rare immunodeficiency disease associated with recombination activating gene.

Additional recommended knowledge

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 (elevation of the white blood cell count)
  • Lymphadenopathy (swelling of one or more lymph nodes)
  • Repeated bacterial infections

Treatment

Omenn syndrome is among the diseases treated with bone marrow transplantation and cord blood stem cells.

 
This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Omenn_syndrome". A list of authors is available in Wikipedia.
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