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

Abstract : 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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https://www.hal.inserm.fr/inserm-00484207
Contributor : Philippe Saas <>
Submitted on : Tuesday, May 18, 2010 - 10:23:52 AM
Last modification on : Tuesday, October 27, 2020 - 2:35:50 PM

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Nicolas Daguindau, Véronique Decot, Rosine Nzietchueng, Christophe Ferrand, Capucine Picard, et al.. Immune constitution monitoring after PBMC transplantation in complete DiGeorge syndrome: an eight-year follow-up.. Clinical Immunology, Elsevier, 2008, 128 (2), pp.164-71. ⟨10.1016/j.clim.2008.03.524⟩. ⟨inserm-00484207⟩

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