Serum-Free Thymus Transplantation in DiGeorge Anomaly

Complete DiGeorge anomaly is a congenital disorder characterized by athymia. Without
successful treatment, patients remain immunodeficient and usually die by age 2 years. In
"typical" complete DiGeorge subjects who have no T cells, thymus transplantation without
immunosuppression has resulted in diverse T cell development and good T cell function. In
"atypical" complete DiGeorge subjects who have no thymus, a rash, and some T cells that
presumably developed extrathymically, thymus transplantation with immunosuppression has
resulted in diverse T cell development and good T cell function. Thus far, thymus
transplantation studies have used thymus cultured in fetal bovine serum (FBS medium). This
protocol's purpose is to determine whether transplanted thymus cultured in serum free medium
can safely support thymopoiesis and T cell reconstitution as does FBS medium cultured thymus
tissue in DiGeorge anomaly subjects. This protocol includes 2 arms: atypical DiGeorge
subjects who will receive immunosuppression and thymus transplantation; and, typical
complete DiGeorge subjects who will receive thymus transplantation without
immunosuppression. Serum free medium use would reduce concerns of animal product exposure
including potential exposure to bovine spongiform encephalopathy(BSE).