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Published on: January 20, 2010
Post-transplant lymphoproliferative disease of the larynx
Nathan D Vandjelovic1,2, Patrick C Barth1,2,3, Stephen P Dunn3,4
1Division of Pediatric Otolaryngology, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.
Insights
Laryngeal post-transplant lymphoproliferative disease (PTLD) is rare in children. Reducing immunosuppression effectively treated airway obstruction in two pediatric liver transplant patients with PTLD.
Area of Science:
- Pediatric medicine
- Transplant surgery
- Oncology
Background:
- Post-transplant lymphoproliferative disease (PTLD) is a rare but serious complication following organ transplantation.
- Laryngeal PTLD, specifically, presents a diagnostic challenge due to its rarity and potential for airway compromise.
Observation:
- Two pediatric liver transplant recipients presented with distinct airway obstruction symptoms.
- Case 1: A 15-month-old with epiglottic and arytenoid infiltrate consistent with polymorphic PTLD.
- Case 2: A 23-month-old with submucosal infiltrate in the epiglottis, arytenoids, post-cricoid region, and uvula, diagnosed as monomorphic PTLD.
Findings:
- Both pediatric patients exhibited symptoms of airway obstruction attributed to PTLD.
- Histopathological examination confirmed polymorphic PTLD in one case and monomorphic PTLD in the other.
- Successful resolution of airway symptoms and findings was achieved by reducing immunosuppression in both patients.
Implications:
- This case series highlights the importance of considering laryngeal PTLD in pediatric transplant recipients presenting with airway obstruction.
- Prompt diagnosis and management, including immunosuppression modulation, are crucial for favorable outcomes.
- Increased clinical suspicion is warranted for PTLD in any post-transplant patient with unexplained airway compromise.
Abstract:
Laryngeal post-transplant lymphoproliferative disease (PTLD) is rare. Here, we describe two pediatric cases. The first, a 15-month-old who underwent liver transplantation at 5 weeks, presented with airway distress. Airway evaluation identified epiglottic and arytenoid infiltrate, and biopsy was consistent with polymorphic PTLD. The second, a 23-month-old who underwent liver transplantation at 13 months, presented with progressive stridor. Airway evaluation revealed sub-mucosal infiltrate of the epiglottis, arytenoids, post-cricoid region, and uvula. Biopsy was consistent with monomorphic PTLD. Airway findings and symptoms resolved for both after immunosuppression reduction. PTLD diagnosis requires a high index of suspicion in post-transplant patients with airway obstruction.
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