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.
Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, USA. marke001@mc.duke.edu
M Louise
ML
Markert
Blythe H
BH
Devlin
Elizabeth A
EA
McCarthy
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