Omenn syndrome in the context of other B cell-negative severe combined immunodeficiencies
A Villa, C Sobacchi, P Vezzoni - IMAJ-RAMAT GAN-, 2002 - ima.org.il
Severe combined immunodeficiencies represent a heterogeneous group of hereditary
defects of the immune system that affect both T and B cells and whose etiology has only
recently begun to be understood. A portion of these SCID patients bear a defect in either of
the two recombination-activating genes, Rag-1 or Rag-2, while others have mutations in a
newly identified gene, Artemis. Omenn syndrome is an unusual severe immunodeficiency
with T cells but no B cells, and peculiar features also due to a defect in Rag-1 or Rag-2 …
defects of the immune system that affect both T and B cells and whose etiology has only
recently begun to be understood. A portion of these SCID patients bear a defect in either of
the two recombination-activating genes, Rag-1 or Rag-2, while others have mutations in a
newly identified gene, Artemis. Omenn syndrome is an unusual severe immunodeficiency
with T cells but no B cells, and peculiar features also due to a defect in Rag-1 or Rag-2 …
