/severe combined immunodeficiency

severe combined immunodeficiency

Rare Disease

MONDO:0015974

Also Known As

SCIDcombined T and B cell inborn immunodeficiencysevere combined immunodeficiencysevere combined immunodeficiency (disease)severe combined immunodeficiency disease

Definition

Severe combined immunodeficiency (SCID) comprises a group of rare monogenic primary immunodeficiency disorders characterized by a lack of functional peripheral T lymphocytes resulting in early-onset severe respiratory infections and failure to thrive. They are classified according to immunological phenotype into SCID with absence of T cells but presence of B cells (T-B+ SCID) or SCID with absence of both (T-B- SCID). Both of these groups include several forms, with or without natural killer (NK) cells.