TCR-alpha-beta-positive T-cell deficiency

Orpha code: 397959OMIM code: 615387

Definition

TCR-alpha-beta-positive T-cell deficiency is 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.

Disease data
Classification

Disease

Synonyms
TCR-alpha-beta+ T-cell deficiency
Niedobór TCR-alfa-beta+ komórek T
ORPHA code
397959
OMIM code
615387
ICD10 code
D81.8
ICD11 code
-

No additional description.

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