Thymus transplantation

• Published in: Clinical immunology (Orlando, Fla.) Vol. 135; no. 2; pp. 236 - 246
• Main Authors: Markert, M. Louise, Devlin, Blythe H., McCarthy, Elizabeth A.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.05.2010
• Subjects: 22q11.2; Allergy and Immunology; Athymia; Cell Count; DiGeorge; DiGeorge Syndrome - immunology; DiGeorge Syndrome - surgery; Humans; Infant; T-Lymphocytes - immunology; T-Lymphocytes - transplantation; Thymus Gland - immunology; Thymus Gland - transplantation; Thymus transplantation; Transplantation, Homologous - adverse effects; Transplantation, Homologous - immunology; Transplantation, Homologous - methods; Treatment Outcome; Athymia; Thymus transplantation; 22q11.2; DiGeorge
• ISSN: 1521-6616; 1521-7035; 1521-7035
• DOI: 10.1016/j.clim.2010.02.007

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.