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Post-transplant lymphoproliferative disorder with supraglottic involvement
Ph Rombaux1, A Marque, M Hamoir
1Department of Otorhinolaryngology, Cliniques Universitaires Saint-Luc, Brussels, Belgium. philippe.rombaux@orlo.ucl.ac.be
B-ENT
|July 8, 2005
Summary
Post-transplant lymphoproliferative disorder (PTLD) can manifest as acute stridor in infants after liver transplants. Prompt diagnosis and reduced immunosuppression are key for remission in these Epstein-Barr virus (EBV) cases.
Area of Science:
- Pediatric Transplantation
- Virology
- Immunology
Background:
- Primary Epstein-Barr virus (EBV) infection post-transplant can lead to post-transplant lymphoproliferative disorder (PTLD).
- Infants, often seronegative at transplant, are at risk for PTLD, with frequent initial manifestations in the ENT area.
Purpose of the Study:
- To report on PTLD with supraglottic involvement in infants following liver transplantation.
- To highlight the diagnostic and management implications of this rare PTLD presentation.
Main Methods:
- Retrospective analysis of two pediatric cases of PTLD with confirmed supraglottic involvement.
- Diagnosis based on pathological and immunological evidence of B-cell proliferation, including EBV detection and LMP1 immunostaining.
Main Results:
- Two infants developed acute stridor 8-10 months post-orthotopic liver transplantation (OLT) due to PTLD.
- Laryngeal examination revealed mucosal hyperplasia; EBV was confirmed. Treatment involved reducing immunosuppression, leading to complete PTLD remission.
Conclusions:
- Acute stridor with supraglottic involvement is a potential PTLD manifestation in pediatric OLT recipients.
- Prompt diagnosis and rapid reduction of immunosuppression are crucial for PTLD management and recovery of immune response against EBV.