Immune constitution monitoring after PBMC transplantation in complete DiGeorge syndrome: an eight-year follow-up.
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.
Daguindau
Nicolas
N
CHU de Nancy, Unité de Thérapie cellulaire et Tissus, Vandoeuvre-lès-Nancy, F-54511-France.
Decot
Véronique
V
Nzietchueng
Rosine
R
Ferrand
Christophe
C
Picard
Capucine
C
Latger-Cannard
Véronique
V
Gregoire
Marie José
MJ
Beri
Mylène
M
Salmon
Alexandra
A
Stoltz
Jean François
JF
Bordigoni
Pierre
P
Bensoussan
Danièle
D
D016516:CD4-CD8 Ratio; D018414:CD8-Positive T-Lymphocytes / Q000737:chemistry / Q000276:immunology; D002678:Chimera; D004247:DNA / Q000235:genetics; D004062:DiGeorge Syndrome / Q000276:immunology* / Q000628:therapy*; D006801:Humans; D007223:Infant; D007963:Leukocytes, Mononuclear / Q000637:transplantation*; D018655:Lymphocyte Count; D008297:Male; D015166:Monitoring, Immunologic; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000276:immunology; D016615:Telomere / Q000737:chemistry; D013950:Thymus Gland / Q000276:immunology; D014185:Transplantation, Isogeneic; D016896:Treatment Outcome
D016693:Receptors, Antigen, T-Cell, alpha-beta; D004247:DNA