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Thymus transplantation for complete DiGeorge syndrome: European experience

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  • E Graham Davies
  • Melissa Cheung
  • Kimberly Gilmour
  • Jesmeen Maimaris
  • Joe Curry
  • Anna Furmanski
  • Neil Sebire
  • Neil Halliday
  • Konstantinos Mengrelis
  • Stuart Adams
  • Jolanta Bernatoniene
  • Ronald Bremner
  • Michael Browning
  • Blythe Devlin
  • Hans Christian Erichsen
  • H Bobby Gaspar
  • Lizzie Hutchison
  • Winnie Ip
  • Marianne Ifversen
  • T Ronan Leahy
  • Elizabeth McCarthy
  • Despina Moshous
  • Kim Neuling
  • Malgorzata Pac
  • Alina Papadopol
  • Kathryn L Parsley
  • Luigi Poliani
  • Ida Ricciardelli
  • David M Sansom
  • Tiia Voor
  • Austen Worth
  • Tessa Crompton
  • M Louise Markert
  • Adrian J Thrasher
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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.

Original languageEnglish
JournalThe Journal of allergy and clinical immunology
Issue number6
Pages (from-to)1660-1670.e16
Publication statusPublished - Dec 2017

    Research areas

  • Journal Article

ID: 52168024