Acute tonsillitis as the first manifestation of post-transplant lymphoproliferative disorder

Johan Nouwen1, Françoise Smets, Philippe Rombaux

  • 1Department of Otolaryngology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.

Insights

Post-transplant lymphoproliferative disorder (PTLD) in children after liver transplants often presents with ear, nose, and throat symptoms. Early tonsillectomy and reduced immunosuppression led to full recovery in all affected patients.

Area of Science:

  • Pediatric Transplantation
  • Immunology
  • Oncology

Background:

  • Post-transplant lymphoproliferative disorder (PTLD) is a serious complication following pediatric liver transplantation.
  • PTLD is associated with Epstein-Barr virus (EBV) infection in immunocompromised children.
  • Early symptoms of PTLD often manifest in the ear, nose, and throat (ENT) region.

Purpose of the Study:

  • To prospectively evaluate the incidence, presentation, and outcomes of PTLD in pediatric liver transplant recipients.
  • To investigate the role of Epstein-Barr virus (EBV) status in the development of PTLD.
  • To assess the effectiveness of tonsillectomy and immunosuppression management for PTLD.

Main Methods:

  • Prospective evaluation of 77 pediatric liver transplant recipients.
  • Monitoring for PTLD development, EBV status, clinical presentation, and treatment response.
  • Analysis of PTLD incidence, localization, pathological findings, and patient evolution.

Main Results:

  • Eight patients (10%) developed PTLD, all with initial ENT symptoms, primarily acute nonbacterial tonsillitis.
  • All PTLD patients were EBV-seronegative pre-transplant, with seroconversion occurring around diagnosis.
  • All patients with tonsillar PTLD treated with tonsillectomy and immunosuppression tapering achieved complete recovery.

Conclusions:

  • Nonbacterial tonsillar inflammation in EBV-infected children post-liver transplant can be an early sign of PTLD.
  • Tonsillectomy combined with judicious immunosuppression tapering is a highly effective treatment for pediatric PTLD.
  • This therapeutic approach offers the best prognosis for complete recovery in children with PTLD.

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