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Thymus transplantation for complete DiGeorge syndrome: European experience. 2017
E Graham Davies, and
Melissa Cheung, and
Kimberly Gilmour, and
Jesmeen Maimaris, and
Joe Curry, and
Anna Furmanski, and
Neil Sebire, and
Neil Halliday, and
Konstantinos Mengrelis, and
Stuart Adams, and
Jolanta Bernatoniene, and
Ronald Bremner, and
Michael Browning, and
Blythe Devlin, and
Hans Christian Erichsen, and
H Bobby Gaspar, and
Lizzie Hutchison, and
Winnie Ip, and
Marianne Ifversen, and
T Ronan Leahy, and
Elizabeth McCarthy, and
Despina Moshous, and
Kim Neuling, and
Malgorzata Pac, and
Alina Papadopol, and
Kathryn L Parsley, and
Luigi Poliani, and
Ida Ricciardelli, and
David M Sansom, and
Tiia Voor, and
Austen Worth, and
Tessa Crompton, and
M Louise Markert, and
Adrian J Thrasher
Infection, Immunity and Inflammation Theme, UCL Great Ormond Street Institute of Child Health, London, United Kingdom; Department of Immunology, Great Ormond Street Hospital, London, United Kingdom. Electronic address: Graham.Davies@gosh.nhs.uk.
BACKGROUND Thymus transplantation is a promising strategy for the treatment of athymic complete DiGeorge syndrome (cDGS). METHODS Twelve patients with cDGS underwent transplantation with allogeneic cultured thymus. OBJECTIVE We sought to confirm and extend the results previously obtained in a single center. RESULTS Two patients died of pre-existing viral infections without having thymopoiesis, and 1 late death occurred from autoimmune thrombocytopenia. One infant had septic shock shortly after transplantation, resulting in graft loss and the need for a second transplant. Evidence of thymopoiesis developed from 5 to 6 months after transplantation in 10 patients. Median circulating naive CD4 counts were 44 × 106/L (range, 11-440 × 106/L) and 200 × 106/L (range, 5-310 × 106/L) at 12 and 24 months after transplantation and T-cell receptor excision circles were 2,238/106 T cells (range, 320-8,807/106 T cells) and 4,184/106 T cells (range, 1,582-24,596/106 T cells). Counts did not usually reach normal levels for age, but patients were able to clear pre-existing infections and those acquired later. At a median of 49 months (range, 22-80 months), 8 have ceased prophylactic antimicrobials, and 5 have ceased immunoglobulin replacement. Histologic confirmation of thymopoiesis was seen in 7 of 11 patients undergoing biopsy of transplanted tissue, including 5 showing full maturation through to the terminal stage of Hassall body formation. Autoimmune regulator expression was also demonstrated. Autoimmune complications were seen in 7 of 12 patients. In 2 patients early transient autoimmune hemolysis settled after treatment and did not recur. The other 5 experienced ongoing autoimmune problems, including thyroiditis (3), hemolysis (1), thrombocytopenia (4), and neutropenia (1). CONCLUSIONS This study confirms the previous reports that thymus transplantation can reconstitute T cells in patients with cDGS but with frequent autoimmune complications in survivors.
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