GvHD-associated cytokine polymorphisms do not associate with Omenn syndrome rather than T− B− SCID in patients with defects in RAG genes
IJ Haq, LJ Steinberg, M Hoenig, M van der Burg… - Clinical …, 2007 - Elsevier
Recombinase activating genes 1/2 (RAG1/2) deficiency, critical to initiate gene
rearrangement encoding lymphocyte receptors, causes T− B− severe combined
immunodeficiency (SCID) and Omenn syndrome (OS), characterised by erythroderma,
hepatosplenomegaly, lymphadenopathy, activated, clonal T cell expansions with restricted
TCRVβ family usage, and opportunistic infection. Many features of OS resemble graft-versus-
host disease (GvHD). Frequency of GvHD-associated cytokine gene polymorphisms (CGPs) …
rearrangement encoding lymphocyte receptors, causes T− B− severe combined
immunodeficiency (SCID) and Omenn syndrome (OS), characterised by erythroderma,
hepatosplenomegaly, lymphadenopathy, activated, clonal T cell expansions with restricted
TCRVβ family usage, and opportunistic infection. Many features of OS resemble graft-versus-
host disease (GvHD). Frequency of GvHD-associated cytokine gene polymorphisms (CGPs) …