Correction of both immunodeficiency and hypoparathyroidism by thymus transplantation in complete DiGeorge syndrome. Issue 5 (6th December 2019)

• Record Type: Journal Article
• Authors: Kreins, Alexandra Y.; Junghanns, Florence; Mifsud, William; Somana, Kathy; Sebire, Neil; Rampling, Dyanne; Worth, Austen; Sirin, Methap; Schuetz, Catharina; Schulz, Ansgar; Hoenig, Manfred; Thrasher, Adrian J.; Davies, Edward G.
• Is Part Of: American journal of transplantation. Volume 20:Issue 5(2020)
• Page Start: 1447
• Page End: 1450
• Publication Date: 2019-12-06
• Subjects: clinical research/practice -- endocrinology/diabetology -- immune deficiency -- immunobiology -- major histocompatibility complex -- thymus/thymic biology -- tolerance: mechanisms -- translational research/science; Transplantation of organs, tissues, etc -- Periodicals; 617.95
• DOI: 10.1111/ajt.15668
• Languages: English
• ISSNs: 1600-6135

Abstract

Abstract : Combined immune deficiency due to athymia in patients with complete DiGeorge syndrome can be corrected by allogeneic thymus transplantation. Hypoparathyroidism is a frequent concomitant clinical problem in these patients, which persists after thymus transplantation. Cotransplantation of allogeneic thymus and parental parathyroid tissue has been attempted but does not achieve durable correction of the patients' hypoparathyroidism due to parathyroid graft rejection. Surprisingly, we observed correction of hypoparathyroidism in one patient after thymus transplantation. Immunohistochemical analysis and fluorescence in situ hybridization confirmed the presence of allogeneic parathyroid tissue in the patient's thymus transplant biopsy. Despite a lack of HLA‐matching between thymus donor and recipient, the reconstituted immune system displays tolerance toward the thymus donor. Therefore we expect this patient's hypoparathyroidism to be permanently cured. It is recognised that ectopic parathyroid tissue is not infrequently found in the thymus. If such thymuses could be identified, we propose that their use would offer a compelling approach to achieving lasting correction of both immunodeficiency and hypoparathyroidism. Abstract : Kreins et al describe a case in which inadvertent transplantation of ectopic parathyroid tissue during a HLA‐mismatched thymus transplant results in enduring correction of hypoparathyroidism in a patient with complete DiGeorge syndrome.