Immune constitution monitoring after PBMC transplantation in complete DiGeorge syndrome: an eight-year follow-up.
Fiche publication
Date publication
août 2008
Auteurs
Membres identifiés du Cancéropôle Est :
Pr BENSOUSSAN Danièle, Dr FERRAND Christophe
Tous les auteurs :
Daguindau N, Decot V, Nzietchueng R, Ferrand C, Picard C, Latger-Cannard V, Gregoire MJ, Beri M, Salmon A, Stoltz JF, Bordigoni P, Bensoussan D
Lien Pubmed
Résumé
A young boy with a confirmed complete DiGeorge Syndrome (cDGS) underwent a peripheral blood mononuclear cell transplantation (PBMCT) from his HLA-identical sister at 4.5 years of age, without a conditioning regimen. Eight years later, he is healthy with good immunological functions in the presence of a stable mixed T-cell chimerism. Absence of recent thymic emigrants is confirmed. We observe an inverted CD4+/CD8+ ratio, related to the CD8 subset expansion, a skewing of the TCR repertoire, especially on the CD8+ subset and a telomere loss on the CD8+ cells compared to the donor. However, these anomalies do not seem to have an impact on functional immunity. PBMCT in cDGS using an HLA-matched sibling donor provides good long-lasting immunity and is an easy alternative to bone marrow transplantation and to thymic transplantation.
Référence
Clin Immunol. 2008 Aug;128(2):164-71