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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

T-cell immune constitution after peripheral blood mononuclear cell transplantation in complete DiGeorge syndrome.

Complete DiGeorge syndrome (cDGS) is a congenital disorder characterized by typical facies, thymic aplasia, susceptibility to infections, hypoparathyroidism and conotruncal cardiac defect. Fetal thymus or post-natal thymus tissue transplantations and human leucocyte antigen (HLA)-genoidentical bone marrow transplantations were followed in a few cases by immune reconstitution. More recently, a peripheral blood mononuclear cell transplantation (PBMCT) was performed with an HLA-genoidentical donor and followed by a partial T-cell engraftment and immune reconstitution. We report a boy with cDGS, without cardiac defect, who suffered recurrent severe infections. At the age of 4 years, he underwent PBMCT from his HLA-genoidentical sister. He received no conditioning regimen, but graft-versus-host disease (GVHD) prophylaxis was with oral cyclosporin A and mycophenolate mofetil. Toxicity was mild, with grade I acute GVHD. The patient is currently 2.5 years post-PBMCT with excellent clinical performances. Mixed chimaerism can only be observed on the T-cell population (50% donor T cells). T-lymphocyte count fluctuated (CD3 more than 400 x 10(6)/l at d 84 and CD4 more than 200 x 10(6)/l at d 46). Exclusive memory phenotype T cells and absence of new thymic emigrants suggest expansion of infused T cells. T-cell mitogen and tetanus antigen responses normalized a few months after transplantation. After immunizations, specific antibodies were produced. PBMCT from an HLA identical sibling could be an efficient treatment of immune deficiency in cDGS.[1]


  1. T-cell immune constitution after peripheral blood mononuclear cell transplantation in complete DiGeorge syndrome. Bensoussan, D., Le Deist, F., Latger-Cannard, V., Grégoire, M.J., Avinens, O., Feugier, P., Bourdon, V., André-Botté, C., Schmitt, C., Jonveaux, P., Eliaou, J.F., Stoltz, J.F., Bordigoni, P. Br. J. Haematol. (2002) [Pubmed]
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