Thymus transplantation in complete DiGeorge anomaly.

[omenn syndrome]

Complete DiGeorge anomaly is characterized by athymia , congenital heart disease , and hypoparathyroidism . This congenital disease is fatal by age 2 years unless immune reconstitution is successful . There are multiple underlying syndromes associated with complete DiGeorge anomaly including 22 q 11 hemizygosity in approximately 50 %, CHARGE association in approximately 25 %, and diabetic embryopathy in approximately 15 %. Approximately one -third of patients present with rash and lymphadenopathy associated with oligoclonal "host " T cells . This condition resembles Omenn syndrome . Immunosuppression is necessary to control the oligoclonal T cells . The results of thymus transplantation are reported for a series of 50 patients , of whom 36 survive . The survivors develop naïve T cells and a diverse T cell repertoire .