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Sinonasal posttransplant lymphoproliferative disorder in pediatric lung transplant patients
Brian W Herrmann1, Stuart C Sweet, David W Molter
1Department of Otorhinolaryngology-Head and Neck Surgery, Washington University School of Medicine, USA. bherrmann@childrensent.com
Insights
Pediatric lung transplant recipients have an increased risk of sinonasal posttransplant lymphoproliferative disorder (PTLD), especially those with cystic fibrosis and nasal polyposis. Close monitoring of the sinonasal region is crucial due to potential asymptomatic presentation.
Area of Science:
- Transplant immunology
- Pediatric oncology
- Otolaryngology
Background:
- Posttransplant lymphoproliferative disorder (PTLD) is a serious complication following organ transplantation.
- Pediatric lung transplant recipients are a vulnerable population for PTLD development.
- Sinonasal PTLD has not been extensively studied in pediatric lung transplant patients.
Purpose of the Study:
- To investigate the occurrence and characteristics of sinonasal PTLD in children who have undergone lung transplantation.
- To identify risk factors associated with sinonasal PTLD in this population.
- To emphasize the importance of surveillance for sinonasal PTLD.
Main Methods:
- Retrospective case series analysis of pediatric lung transplant recipients.
- Inclusion criteria: patients under 18 years old with PTLD post-pulmonary transplantation.
- Data collection period: January 1, 1990, to December 31, 2003.
Main Results:
- Eight out of 32 PTLD cases (25%) occurred in the head and neck region.
- Sinonasal PTLD was the most frequent head and neck manifestation (63%).
- Forty percent of sinonasal PTLD cases were asymptomatic (occult disease).
- A notable association was observed between sinonasal PTLD and pre-existing nasal polyposis in cystic fibrosis patients (P = 0.07).
Conclusions:
- This study highlights an increased frequency of sinonasal PTLD in pediatric lung transplant recipients.
- Children with cystic fibrosis and associated nasal polyposis appear to be at higher risk.
- Routine surveillance of the sinonasal region is recommended for early detection of PTLD, given its potential for asymptomatic presentation.
Objective:
To evaluate sinonasal manifestations of posttransplant lymphoproliferative disorder (PTLD) in the pediatric lung transplant population.
Study Design And Methods:
Case series of children less than 18 years presenting with PTLD after pulmonary transplantation at St Louis Children's Hospital between Jan 1, 1990 and Dec 31, 2003.
Results:
Two hundred eighty-two lung transplants were performed in 246 children. Thirty-two cases of histopathologically confirmed PTLD were identified with 8 (25%) presenting in the head and neck. Sinonasal PTLD was the most common site of head and neck involvement (63%), with 40% of patients presenting with occult disease. All patients with sinonasal PTLD had longstanding nasal polyposis related to cystic fibrosis (P = 0.07).
Conclusions:
This is, to our knowledge, the first report identifying an increased frequency of sinonasal PTLD after pediatric pulmonary transplantation, particularly in children with cystic fibrosis and associated nasal polyposis. Because sinonasal PTLD may be asymptomatic, this region should receive close scrutiny on surveillance evaluations.
