Related Experiment Video
Updated: Jun 8, 2025
Expression of Exogenous Cytokine in Patient-derived Xenografts via Injection with a Cytokine-transduced Stromal Cell Line
Published on: May 10, 2017
Posttransplant Lymphoproliferative Disorder in Pediatric Solid-Organ Transplant Recipients: A 7-Year Single-Center
Fatma Burcu Belen Apak1, Pamir Işik, Lale Olcay
1From the Department of Pediatric Hematology and Oncology, Baskent University Medical Faculty, Ankara, Turkey.
Insights
Pediatric posttransplant lymphoproliferative disorder (PTLD) can be effectively treated with rituximab and reduced immunosuppression, leading to complete remission in all studied cases. Early diagnosis and monitoring of Epstein-Barr virus are crucial for managing PTLD in pediatric solid-organ transplant recipients.
Area of Science:
- Pediatric Transplant Medicine
- Oncology
- Immunology
Background:
- Posttransplant lymphoproliferative disorder (PTLD) is a serious complication after solid-organ transplantation, often linked to Epstein-Barr virus (EBV).
- Understanding PTLD incidence, clinical features, and outcomes in pediatric populations is vital for improving patient care.
Purpose of the Study:
- To analyze the incidence, clinical characteristics, and treatment outcomes of pediatric PTLD at a single center.
- To evaluate the effectiveness of current treatment strategies, including rituximab and immunosuppression management.
- To emphasize the importance of early diagnosis and EBV monitoring in pediatric solid-organ transplant recipients.
Main Methods:
- Retrospective analysis of medical records for pediatric liver and kidney transplant recipients from 2017 to 2024.
- Identification of PTLD cases through pathology-confirmed lymphadenopathy or tumors.
- Categorization of PTLD into benign and malignant groups, with early PTLD defined within one year post-transplant.
- Assessment of EBV association using in situ hybridization.
Main Results:
- Ten pediatric patients (9 liver, 1 kidney) were diagnosed with PTLD over 7 years, an incidence of 8.7% in pediatric liver transplants.
- Common symptoms included fever, lymphadenopathy, and hepatosplenomegaly; mean age at diagnosis was 46.4 months.
- All patients achieved complete remission with treatments including rituximab, immunosuppression reduction, IVIG, and chemotherapy, with a mean follow-up of 22.9 months.
Conclusions:
- Rituximab combined with immunosuppression reduction is an effective strategy for achieving complete remission in pediatric PTLD, especially in early polymorphic cases.
- Despite challenges, all patients in the study achieved remission, indicating improved outcomes for pediatric PTLD.
- Active monitoring for EBV and early PTLD diagnosis are critical for reducing complications in pediatric solid-organ transplant recipients.
Objectives:
Posttransplant lymphoproliferative disorder is a consequential complication following solid-organ transplant, particularly associated with the Epstein-Barr virus. We studied a single center's cases of pediatric posttransplant lymphoproliferative disorder for a 7-year period and focused on incidence rates, anatomic sites involved, and correlation with clinical outcomes. We explored clinical features and treatment outcomes in patients with pediatric posttransplant lymphoproliferative disorder, with emphasis on patient survival and associated clinical ramifications.
Materials And Methods:
This was a retrospective analysis of medical records from pediatric solid-organ transplant recipients (liver or kidney) at Baskent University Ankara Hospital Organ Transplantation Center between January 1, 2017, and January 1, 2024, approved by the Institutional Review Board (KA24/63). We identified cases based on pathology-confirmed persistent lymphadenopathy or tumorous lesions. Patient categorization distinguished between malignant and benign groups. Early posttransplant lymphoproliferative disorder was defined within the initial year after transplant. Epstein?Barr virus association was determined through in situ hybridization, and patient characteristics were reviewed comprehensively.
Results:
In 7 years, 10 pediatric patients (9 liver transplants, 1 kidney transplant) were diagnosed with posttransplant lymphoproliferative disorder, with an incidence of 8.7% for pediatric liver transplants. Mean age at diagnosis was 46.4 months, and mean time from transplant to diagnosis was 21.2 months. The most common complaints at diagnosis included fever, lymphadenopathy, hepatosplenomegaly, dyspnea, and diarrhea. Treatment modalities included rituximab, immunosuppression reduction, intravenous immunoglobulin therapy, and chemotherapy (NHL Berlin-Frankfurt-Münster 90 protocols). All patients achieved remission (mean follow-up, 22.9 mo).
Conclusions:
Early diagnosis of posttransplant lymphoproliferative disorder is important, and rituximab with immunosuppression reduction is effective to achieve complete remission, particularly in early polymorphic cases. Despite challenges, all patients achieved remission, signaling improved outcomes in pediatric posttransplant lymphoproliferative disorder. Active monitoring of Epstein?Barr virus infection may further reduce posttransplant lymphoproliferative disorder complications in pediatric solid-organ transplant; hence, early diagnosis is crucial.
