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Immune constitution monitoring after PBMC transplantation in complete DiGeorge syndrome: an eight-year follow-up.

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Daguindau, Nicolas | Decot, Véronique | Nzietchueng, Rosine | Ferrand, Christophe | Picard, Capucine | Latger-Cannard, Véronique | Gregoire, Marie José | Beri, Mylène | Salmon, Alexandra | Stoltz, Jean François | Bordigoni, Pierre | Bensoussan, Danièle

Edité par CCSD ; Elsevier -

International audience. 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.

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