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Published on: May 10, 2017
Posttransplantation lymphoproliferative disorders in pediatric thoracic organ recipients
G J Boyle1, M G Michaels, S A Webber
1Department of Pediatrics, Children's Hospital of Pittsburgh, University of Pittsburgh, Pennsylvania, USA.
Insights
Posttransplantation lymphoproliferative disorders (PTLD) occur more frequently in pediatric thoracic organ transplant recipients. Primary Epstein-Barr virus (EBV) infection post-transplant is a major risk factor for PTLD development.
Area of Science:
- Pediatric transplantation medicine
- Immunology
- Oncology
Background:
- Posttransplantation lymphoproliferative disorders (PTLD) are a significant concern in organ transplant recipients.
- Understanding PTLD in pediatric thoracic organ transplant recipients is crucial for improving outcomes.
Purpose of the Study:
- To investigate the incidence, risk factors, clinical features, and outcomes of PTLD in children undergoing thoracic organ transplantation.
- To identify key associations, such as Epstein-Barr virus (EBV) status, with PTLD development.
Main Methods:
- Retrospective analysis of medical records for 120 pediatric patients surviving over one month post-thoracic organ transplant.
- Review of PTLD diagnosis, clinical presentation, treatment strategies, and patient outcomes.
Main Results:
- PTLD occurred in 11.7% of pediatric thoracic organ transplant recipients, with higher rates in lung/heart-lung recipients (19.5%) compared to heart recipients (7.7%).
- Clinical presentations varied widely, from asymptomatic nodules to multiorgan failure. Primary EBV infection post-transplant was significantly associated with PTLD.
- Treatment involving reduced immunosuppression and antiviral therapy led to PTLD resolution in eight patients; five deaths were attributed to PTLD.
Conclusions:
- PTLD is more frequent in pediatric thoracic organ transplant recipients than in adults.
- Primary EBV infection following transplantation is the primary risk factor for PTLD.
- Management should include immunosuppression reduction and vigilant surveillance for PTLD in EBV-seronegative patients post-transplant.
Objective:
To determine the frequency, predisposing factors, clinical presentation, and outcome of posttransplantation lymphoproliferative disorders (PTLDs) in pediatric thoracic organ transplant recipients.
Methods:
Retrospective review of the medical records of all 120 children who survived longer than 1 month after thoracic organ transplantation at our center.
Results:
PTLD was diagnosed in 14 patients (11.7%), including 7.7% of heart and 19.5% of heart-lung/lung recipients. Presentation of PTLD was variable, ranging from asymptomatic lung nodules on chest radiograph to diffuse multiorgan failure. Treatment with a reduction of immunosuppression and antiviral therapy resulted in resolution of PTLD in eight patients. Eight patients died. PTLD contributed to death in five. No patient seropositive for Epstein-Barr virus (EBV) before transplantation had PTLD. There was a significant association between primary EBV infection after transplantation and the presence of PTLD.
Conclusions:
PTLD occurs with greater frequency in pediatric thoracic organ transplant recipients than in the adult transplant population. Primary EBV infection after transplantation is the major risk factor for the development of PTLD. Patients in whom primary EBV infection develops after transplantation should be managed with a reduction in immunosuppression and with heightened surveillance for the development of PTLD.
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