TCR-alpha-beta-positive T-cell deficiency
TCR-alpha-beta-positive T-cell deficiency
Definition
A non-severe combined immunodeficiency disorder manifesting with recurrent respiratory infections, candidiasis, diarrhea, and failure to thrive. Patients show a clear predisposition to herpes viral infections, and features of immune dysregulation, including hypereosinophilia, vitiligo, and alopecia areata. Other features include lymphadenopathy and hepatosplenomegaly. CD3+ T-cells express TCR- gamma|delta, but little or no TCR-alpha|beta.
Also known as IMD7, TCR-alpha-beta+ T-cell deficiency — per MONDO