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Published on: November 10, 2023
Post-transplant lymphoproliferative disorder of the pediatric airway: Presentation and management
Allison F O'Neill1, Eelam A Adil2, Alexandria L Irace3
1Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA.
Insights
Post-transplant lymphoproliferative disorder (PTLD) in pediatric airway cases is often Epstein-Barr virus (EBV)-associated. Early diagnosis and management, including immunosuppression reduction and therapies like rituximab, are crucial for airway compromise resolution.
Area of Science:
- Oncology
- Immunology
- Pediatrics
Background:
- Post-transplant lymphoproliferative disorder (PTLD) is a rare but serious complication following organ transplantation, particularly in pediatric patients.
- Immunosuppression therapy, necessary to prevent graft rejection, increases the risk of PTLD.
- There is a lack of established consensus regarding the optimal evaluation and management strategies for PTLD, especially in pediatric cases.
Observation:
- A pediatric patient with a history of hepatoblastoma developed laryngo-tracheal PTLD after a liver transplant.
- Diagnostic imaging, including PET/CT scans, revealed abnormal fluorodeoxyglucose uptake in the larynx, esophagus, and lymph nodes.
- Laryngeal biopsy confirmed Epstein-Barr virus (EBV)-positive PTLD.
Findings:
- PTLD affecting the pediatric airway is frequently associated with EBV.
- Patients may present with nonspecific symptoms, but can rapidly develop significant airway compromise.
- A combination of peripheral blood PCR, biopsy, and PET/CT imaging is essential for diagnosis.
- Treatment involving rituximab and reduced immunosuppression led to disease resolution in the described case.
Implications:
- High index of suspicion is necessary for early detection of pediatric airway PTLD.
- Multidisciplinary management involving reduction of immunosuppression and systemic therapies is recommended.
- Further research is needed to establish standardized diagnostic and therapeutic guidelines for pediatric PTLD of the airway.
Objective:
Post-transplant lymphoproliferative disorder (PTLD) is a rare complication of immunosuppression with little consensus on its evaluation and management. The purpose of this contemporary review is to describe a pediatric patient with PTLD of the airway and review the literature to provide multidisciplinary recommendations regarding management.
Data Sources:
Retrospective chart and literature review.
Review Methods:
A pediatric patient with PTLD of the airway is described. An extensive literature search to review the existing data on pediatric PTLD of the upper airway was also performed.
Results:
A pediatric patient with mixed fetal/embryonal hepatoblastoma developed laryngo-tracheal PTLD following liver transplantation. Diagnostic positron emission tomography (PET) scan demonstrated multiple sites of abnormal fluorodeoxyglucose (FDG) uptake within the larynx, distal esophagus, cervical lymph nodes, and abdomen concerning for PTLD. Laryngeal biopsy demonstrated Epstein-Barr virus (EBV) positive cells confirming the diagnosis. Rituximab therapy and reduction of immunosuppression resulted in resolution of his laryngeal disease in 3 months. An extensive literature search to review the existing data on pediatric PTLD of the larynx and trachea revealed 14 reported cases.
Conclusions:
PTLD of the pediatric airway is an EBV-associated disease that requires a high index of suspicion as patients can often present with non-specific signs and symptoms but progress to have significant airway compromise. Evaluation consists of peripheral blood polymerase chain reaction (PCR) assays, biopsy, and PET/CT imaging. Management options include reduction of immunosuppression and/or systemic therapies.
