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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.
Abstract:
Post-transplant lymphoproliferative disorder (PTLD) is a life-threatening complication that may follow orthotopic liver transplantation (OLT) in children. The first symptoms are often in the ear, nose, or throat (ENT) area. This abnormal proliferation of lymphoid cells is related to Epstein-Barr virus (EBV) infection in immunocompromised children. The incidence of PTLD, EBV status before OLT and at the diagnosis of PTLD, delay between OLT and PTLD, localization, pathological investigations, and the treatment and evolution of PTLD were prospectively evaluated in 77 pediatric liver transplant recipients. Eight patients (10%) developed PTLD, all with an ENT presentation. Seven had acute nonbacterial tonsillitis (with a negative throat swab), and 1 had a pharyngolaryngeal localization at the time of the diagnosis. Four patients had associated involvement outside the ENT area. All patients were EBV-seronegative at the time of OLT; 6 underwent seroconversion at the time of diagnosis, and 2 within 9 and 20 months of diagnosis. All patients presented with low-grade PTLD. All patients with acute tonsillitis associated with EBV seroconversion underwent immediate tonsillectomy, and immunosuppression was decreased as much as tolerated. This therapeutic protocol led to complete recovery in all patients. After OLT in children, nonbacterial tonsillar inflammation or hypertrophy associated with an EBV infection is often the first manifestation of PTLD. Tonsillectomy combined with tapering of immunosuppression offers the best chance for a complete recovery.