label: IL-7R-Alpha-deficient Severe Combined Immunodeficiency
label_lc: il-7r-alpha-deficient severe combined immunodeficiency
description: An autosomal recessive form of severe combined immunodeficiency characterized as T-cell negative, B-cell positive, NK cell positive, and with mutations in the gene encoding interleukin-7 receptor alpha chain protein. This class of SCID accounts for approximately 10% of all SCID cases.