TCR-alpha-beta-positive T-cell deficiency
Engelsk navn: TCR-alpha-beta-positive T-cell deficiency
Engelske synonym: TCR-alpha-beta+ T-cell deficiency
Definisjon
A rare, hereditary primary immunodeficiency characterized by recurrent respiratory tract infection, otitis media, candidiasis, diarrhea, as well as various signs and symptoms of immune dysregulation (hypereosinophilia, eczema, vitiligo, alopecia areata, autoimmune hemolytic anemia, pityriasis rubra pilaris). Failure to thrive, moderate lymphadenopathy and hepatomegaly have also been reported.
Fra Orphanet
ORPHA: 397959
Klassifiseringsnivå: Disorder
ICD-10: D81.8