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Published on: January 13, 2023
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
Insights
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.
Objective:
Transplant patients with primary Epstein-Barr virus (EBV) infection may develop post-transplant lymphoproliferative disorder (PTLD). Since many infants are seronegative at the time of transplantation, PTLD is a major concern for paediatric transplant centres. First manifestations of PTLD are frequently observed in the ENT area with adenoidal and/or tonsillar involvement.
Design:
Retrospective study of two cases of PTLD with confirmed supraglottic involvement, their management and outcome. Only patients with pathologically and immunologically demonstrated B-cell proliferation were diagnosed as PTLD.
Result:
Two infants developed an acute stridor during PTLD respectively 8 and 10 months after orthotopic liver transplantation (OLT). These infants were seronegative for EBV at the time of transplantation. IgM anti-EBV and/or detection of EBV genome by polymerase chain reaction were positive. Laryngeal examination revealed hypopharyngeal and/or supraglottic mucosal hyperplasia. Immunostaining of laryngeal biopsy was positive for latent membrane protein-1 (LMP1). Patients were treated by a reduction in immunosuppression as far as tolerated with the intent to recover natural immune response by the patient over the proliferation of EBV-infected cells. Complete remission of PTLD was observed in these two cases.
Conclusion:
Tonsillar hypertrophy and adenoid enlargement are the most encountered features of PTLD in OLT occurring in the ENT area. Acute stridor with supraglottic involvement may also be observed in PTLD and must be promptly diagnosed as the prognosis of this disorder is related to rapid reduction in immunosuppression and consequently to the recovering of a natural immune response against the EBV infection.
