Immunologic reconstitution in 22q deletion (DiGeorge) syndrome.
Adoptive transfer of mature T cells (ATMTC) through bone marrow (BM) transplantation, first attempted over 20 years ago, has recently emerged as a successful therapy for complete 22q deletion syndrome (22qDS). This provides a potential option to thymic transplantation (TT) for immune reconstitution in 22qDS. Compared to thymic transplant, ATMTC is an easier procedure to accomplish and is available at more centers. However, there are differences in the nature of the T-cell reconstitution that results. Predictably, more naïve T cells and recent thymic emigrants are present in patients treated with thymus transplant. There are no significant differences in mortality between the two procedures, but the number of patients is too limited to conclude that the procedures are equally effective. Adoptive transfer should be pursued as a reasonable treatment for 22qDS patients requiring immune reconstitution when thymus transplant is not available.
McGhee
Sean A
SA
Department of Pediatrics, Division of Pediatric Immunology, David Geffen School of Medicine at UCLA, MDCC 12-430, Los Angeles, CA 90095-1752, USA.
Lloret
Maria Garcia
MG
Stiehm
E Richard
ER
D019264:Adoptive Transfer; D015703:Antigens, CD / Q000096:biosynthesis; D016026:Bone Marrow Transplantation / Q000401:mortality; D002452:Cell Count; D002892:Chromosomes, Human, Pair 22; D004062:DiGeorge Syndrome / Q000235:genetics / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D005500:Follow-Up Studies; D006801:Humans; D007167:Immunotherapy; D016019:Survival Analysis; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology / Q000637:transplantation*; D013950:Thymus Gland / Q000276:immunology / Q000473:pathology / Q000637:transplantation*; D016896:Treatment Outcome
D015703:Antigens, CD