Thymus Transplantation for Complete Digeorge Syndrome: European Experience (original) (raw)
2017, The Journal of allergy and clinical immunology
Thymus transplantation is a promising strategy for the treatment of athymic complete DiGeorge syndrome (cDGS). Twelve patients with cDGS were transplanted with allogeneic cultured thymus. To confirm and extend the results previously obtained in a single centre. Two patients died of pre-existing viral infections without developing thymopoeisis and one late death occurred from autoimmune thrombocytopaenia. One infant suffered septic shock shortly after transplant resulting in graft loss and the need for a second transplant. Evidence of thymopoeisis developed from 5-6 months after transplantation in ten patients. The median (range) of circulating naïve CD4 counts (x10(6)/L) were 44(11-440) and 200(5-310) at twelve and twenty-four months post-transplant and T-cell receptor excision circles were 2238 (320-8807) and 4184 (1582 -24596) per10(6) T-cells. Counts did not usually reach normal levels for age but patients were able to clear pre-existing and later-acquired infections. At a median...
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