X-linked SCID(γc-chain deficiency)

Male infants with XSCID appear normal at birth. Clinical features of XSCID contain failure to thrive, oral trush, candidal diaper rash, absent tonsils, persistence of infection despite conventional treatment. Other features include presence of lymphocytopenia ('alymphocytosis'), earlier age at death, vulnerability to viral and fungal and bacterial infections, lack of delayed hypersensitivity, atrophy of the thymus, and lack of benefit by gamma globulin administration. Patients with X-SCID have extreme susceptibility to infections.

Therapeutic options