Post-transplant lymphoproliferative disorder with supraglottic involvement
Ph. Rombaux*, A. Marque*, M. Hamoir*, F. Smets** and E. Sokal**
*Department of Otorhinolaryngology, Cliniques Universitaires Saint-Luc; **Department of Pediatrics, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
Post transplant lymphoproliferative disorder; supraglottic area
53 - 56
Post-transplant lymphoproliferative disorder with supraglottic involvement. 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 cen- tres. 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 out- come. 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 trans- plantation (OLT). These infants were seronegative for EBV at the time of transplantation. IgM anti-EBV and/or detec- tion 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 occur- ring 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.
Vol. 1, 2005, 1st trimester

Post-transplant lymphoproliferative disorder with supraglottic involvement