Deficiency of CD3 delta on T cells is a rare but serious form of immunodeficiency.
The CD3 antigen/T cell receptor complex normally contains alpha, beta, delta, epsilon and gamma proteins.
Inheritance: autosomal recessive (AR)
Deficiency in CD3 delta:
(1) It is associated with a severe combined immunodeficiency (SCID).
(2) The clinical onset is during infancy with severe viral infections.
(3) The patient may show features of OMENN Syndrome (another type of SCID, see above).
(4) The condition is associated with (a) markedly decreased T cells, (b) normal numbers of B and NK cells, and (c) decreased serum immunoglobulins.
(5) The condition is fatal unless a matched stem cell transplant can be performed.
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Specialty: Immunology/Rheumatology, Infectious Diseases
