Thymus transplantation.
Thymus transplantation is a promising investigational therapy for infants born with no thymus. Because of the athymia, these infants lack T cell development and have a severe primary immunodeficiency. Although thymic hypoplasia or aplasia is characteristic of DiGeorge anomaly, in "complete" DiGeorge anomaly, there is no detectable thymus as determined by the absence of naive (CD45RA(+), CD62L(+)) T cells. Transplantation of postnatal allogeneic cultured thymus tissue was performed in sixty subjects with complete DiGeorge anomaly who were under the age of 2 years. Recipient survival was over 70%. Naive T cells developed 3-5 months after transplantation. The graft recipients were able to discontinue antibiotic prophylaxis, and immunoglobulin replacement. Immunosuppression was used in a subset of subjects but was discontinued when naive T cells developed. The adverse events have been acceptable with thyroid disease being the most common. Research continues on mechanisms underlying immune reconstitution after thymus transplantation.
Markert
M Louise
ML
Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, USA. marke001@mc.duke.edu
Devlin
Blythe H
BH
McCarthy
Elizabeth A
EA
D002452:Cell Count; D004062:DiGeorge Syndrome / Q000276:immunology / Q000601:surgery*; D006801:Humans; D007223:Infant; D013601:T-Lymphocytes / Q000276:immunology* / Q000637:transplantation; D013950:Thymus Gland / Q000276:immunology / Q000637:transplantation*; D014184:Transplantation, Homologous / Q000009:adverse effects / Q000276:immunology / Q000379:methods; D016896:Treatment Outcome