Severe Combined Immunodeficiency Due to a Specific Defect in the Production of Interleukin-2

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Abstract
SEVERE combined immunodeficiency (SCID) syndrome is caused by a group of genetic diseases characterized by abnormal function of T and B lymphocytes.1 SCID was first described in children with lymphopenia, agammaglobulinemia, and absent proliferative responses to T-lymphocyte mitogens, such as phytohemagglutinin. Since the original description of X-linked lymphopenic SCID (the so-called Swiss type of agammaglobulinemia), other children have been found to have SCID due to the absence of the enzyme adenosine deaminase, which is required for the development of T lymphocytes. Together, the X-linked and adenosine deaminase—deficient forms of SCID account for approximately 40 to 50 percent of all cases.