Severe combined immunodeficiency
Også kjent som: Alvorlig kombinert immunsvikt, SCID
Engelsk navn: Severe combined immunodeficiency
Engelske synonym: SCID
Definisjon
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) (see these terms). Both of these groups include several forms, with or without natural killer (NK) cells.
Fra Orphanet
ORPHA: 183660
Klassifiseringsnivå: Group of disorders
ICD-10: