Post-transplant lymphoproliferative disorder presenting as supraglottitis following pediatric heart transplantation treated with EBV-specific cytotoxic T-lymphocytes

We present the case of a 4 year-old boy post heart transplantation who presented with signs and symptoms of critical airway obstruction and was initially diagnosed with infective supraglottitis. Following re-presentation and biopsy, this was confirmed as post-transplant lymphoproliferative disorder (PTLD) in an unusual site; laryngeal PTLD is rare. The patient failed standard therapy and ultimately was successfully treated with EBV-specific cytotoxic T lymphocytes (CTL). This case describes a rare presentation of PTLD which required a novel treatment approach including elective tracheostomy prior to CTL therapy. The treatment was successful and the patient was decannulated prior to discharge following 4 negative biopsies, the most recent 6 months following treatment completion. The case also highlights the importance of extra-vigilance in the post-transplant population and of a collaborative approach between multiple specialties across two separate countries including the transplant center and the referral center. J Heart Lung Transplant 2022;41:708- 711 (c) 2022 The Author(s). Published by Elsevier Inc. on behalf of International Society for Heart and Lung Transplantation. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/)

Automated summary

This case describes a rare presentation of post-transplant lymphoproliferative disorder (PTLD) in a 4 year-old boy who had undergone heart transplantation. The PTLD developed in an unusual site, the larynx, and required a novel treatment approach including elective tracheostomy and EBV-specific cytotoxic T lymphocytes (CTL) therapy. The treatment was successful and the patient achieved negative biopsy results, allowing for decannulation and discharge.