Severe combined immunodeficiency

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.

Also known as SCID, severe combined immunodeficiency, severe combined immunodeficiency (disease), severe combined immunodeficiency disease — per MONDO

Also identified as

• DOID 627 per MONDO
• MESH D016511 per MONDO
• NCIT C3472 per MONDO
• Orphanet 183660 per MONDO
• SCTID 31323000 per MONDO
• UMLS C0085110 per MONDO

Drugs indicated