{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T08:40:27Z","timestamp":1761986427420},"reference-count":41,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2002,6,10]],"date-time":"2002-06-10T00:00:00Z","timestamp":1023667200000},"content-version":"vor","delay-in-days":9,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2002,6]]},"abstract":"<jats:p><jats:bold>Summary.\u2002<\/jats:bold> 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\u2010natal thymus tissue transplantations and human leucocyte antigen (HLA)\u2010genoidentical 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\u2010genoidentical donor and followed by a partial T\u2010cell engraftment and immune reconstitution. We report a boy with cDGS, without cardiac defect, who suffered recurrent severe infections. At the age of 4\u2003years, he underwent PBMCT from his HLA\u2010genoidentical sister. He received no conditioning regimen, but graft\u2010versus\u2010host disease (GVHD) prophylaxis was with oral cyclosporin A and mycophenolate mofetil. Toxicity was mild, with grade I acute GVHD. The patient is currently 2\u00b75\u2003years post\u2010PBMCT with excellent clinical performances. Mixed chimaerism can only be observed on the T\u2010cell population (50% donor T cells). T\u2010lymphocyte count fluctuated (CD3 more than 400\u2003\u00d7\u200310<jats:sup>6<\/jats:sup>\/l at d\u200384 and CD4 more than 200\u2003\u00d7\u200310<jats:sup>6<\/jats:sup>\/l at d\u200346). Exclusive memory phenotype T cells and absence of new thymic emigrants suggest expansion of infused T cells. T\u2010cell 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.<\/jats:p>","DOI":"10.1046\/j.1365-2141.2002.03496.x","type":"journal-article","created":{"date-parts":[[2003,3,11]],"date-time":"2003-03-11T06:45:37Z","timestamp":1047365137000},"page":"899-906","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["T\u2010cell immune constitution after peripheral blood mononuclear cell transplantation in complete DiGeorge syndrome"],"prefix":"10.1111","volume":"117","author":[{"given":"Dani\u00e8le","family":"Bensoussan","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fran\u00e7oise","family":"Le 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