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Nondestructive versus advanced post-transplant lymphoproliferative disorder in a single-center pediatric kidney
Ellen Wu1, Joo-Young Lee1, Ian Gelarden2
1Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
Insights
Nondestructive post-transplant lymphoproliferative disorders (PTLD) in pediatric kidney transplant patients often present with sleep-disordered breathing and can be managed conservatively. Further research is needed for this understudied group.
Area of Science:
- Pediatric Nephrology
- Transplantation Immunology
- Oncology
Background:
- Pediatric kidney transplant recipients face risks for post-transplant lymphoproliferative disorders (PTLD).
- PTLD presents a spectrum from nondestructive to destructive lesions.
- Current evidence for managing nondestructive PTLD is insufficient.
Purpose of the Study:
- To describe clinical characteristics, presentation, and management of nondestructive versus advanced PTLD in pediatric kidney transplant recipients.
- To evaluate outcomes of conservative management for nondestructive PTLD.
Main Methods:
- A single-center case series from January 1, 2008, to December 31, 2019.
- Inclusion of pediatric kidney transplant recipients diagnosed with PTLD.
- Comparison of clinical features and management between nondestructive and advanced PTLD.
Main Results:
- Eighteen patients had nondestructive PTLD; seven had advanced PTLD.
- Nondestructive PTLD often presented with tonsillar hypertrophy/snoring (66.7%) and was managed conservatively.
- Advanced PTLD patients presented with fever, elevated creatinine, new masses, or GI symptoms, requiring imaging, oncology consultation, and chemotherapy.
Conclusions:
- Nondestructive PTLD in pediatric kidney transplant recipients frequently manifests with sleep-disordered breathing symptoms.
- Conservative management of nondestructive PTLD yields excellent clinical outcomes.
- Further research is essential to guide the care of pediatric patients with nondestructive PTLD.
Background:
Pediatric kidney transplant recipients are at risk for the development of post-transplant lymphoproliferative disorders (PTLD), a group of potentially devastating diseases that present on a spectrum of severity ranging from nondestructive PTLD to more histologically destructive lesions. Currently, there is inadequate evidence to guide evaluation and management of nondestructive PTLD.
Methods:
This is a single-center case series of pediatric kidney transplant recipients between January 1, 2008 and December 31, 2019, who were diagnosed with PTLD. The aim was to describe clinical characteristics, presentation, and management of nondestructive versus advanced PTLD.
Results:
Eighteen patients were diagnosed with nondestructive PTLD and seven with more advanced PTLD histopathology. The majority (66.7%) of nondestructive PTLD patients (n = 16) presented with tonsillar hypertrophy and/or snoring and were managed conservatively, with minimal reduction in tacrolimus dose and no further evaluation. No patient progressed to more advanced PTLD. Advanced PTLD patients (n = 7) were more likely to present with fever, elevated creatinine, a new mass of gastrointestinal symptoms. They received workup with imaging and oncology consultation, and were managed with chemotherapy.
Conclusions:
Patients with nondestructive PTLD often present with symptoms of sleep-disordered breathing and can be managed conservatively with excellent clinical outcomes. More study is needed to guide care of this under-researched population.
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