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Post-transplantation lymphoproliferative disease in pediatric liver recipients in Taiwan
Chien-Ting Hsu1, Mei-Hwei Chang1, Ming-Chih Ho2
1Department of Pediatrics, National Taiwan University Hospital, No. 8, Chung-Shan S. Rd., Taipei, Taiwan.
Insights
Post-transplantation lymphoproliferative disorder (PTLD) is a serious complication after pediatric liver transplants. While most children survive with treatment, advanced stage IV disease significantly worsens prognosis, and chronic rejection or graft failure can occur.
Area of Science:
- Pediatric Oncology
- Transplant Immunology
- Hematology
Background:
- Post-transplantation lymphoproliferative disorder (PTLD) is a significant and potentially fatal complication following liver transplantation (LT) in children.
- Understanding PTLD's characteristics and outcomes is crucial for improving pediatric transplant care.
Purpose of the Study:
- To investigate the disease characteristics, treatment outcomes, and prognostic factors of PTLD in pediatric liver transplant recipients.
- To identify key factors influencing survival and complications in pediatric PTLD.
Main Methods:
- A retrospective observational study analyzed 16 pediatric liver transplant recipients diagnosed with PTLD between 2001 and 2013.
- Data collected included disease onset, location, histopathology, treatment, survival, and complications.
Main Results:
- PTLD occurred at a median of 8 months post-LT, with 62.5% presenting early (<1 year).
- Extranodal involvement (81.3%) and gastrointestinal tract (68.8%) were common. Histopathology varied, including polymorphic PTLD and diffuse large B-cell lymphoma.
- Overall 1- and 5-year survival rates were 87.5% and 79.5%, respectively. Stage IV disease (HR=13.37, P=0.037) was linked to poor survival.
Conclusions:
- PTLD is a major complication in pediatric LT, but effective treatment strategies can lead to long-term survival.
- Stage IV PTLD is a critical risk factor for poor outcomes in pediatric patients.
- Monitoring for chronic rejection and graft failure is essential during PTLD management.
Background:
Post-transplantation lymphoproliferative disorder (PTLD) is a heterogeneous, potentially life-threatening complication after liver transplantation in children. In this study, the disease characteristics, outcomes, and prognostic factors of PTLD were investigated.
Methods:
A retrospective, observational study was conducted on 16 pediatric liver transplant (LT) recipients who developed PTLD between February 2001 and December 2013 at a tertiary referral center in Taiwan. The disease characteristics and treatment outcomes of the patients were analyzed.
Results:
The median time from liver transplantation to the development of PTLD was 8 months. Early onset (<1 year post-transplantation) PTLD constituted 62.5% of the cases. PTLD location was frequently extranodal (81.3%) and mostly involved the gastrointestinal tract (68.8%). Histopathologic findings showed polymorphic PTLD in six cases (37.5%), diffuse large B-cell lymphoma in five cases (31.3%), and Burkitt's PTLD in two cases (12.5%). Early lesions in PTLD, T-cell lymphoma, and Hodgkin's lymphoma were observed in one case each (6.3%). Overall survival at 1- and 5-years post-PTLD diagnosis was 87.5% and 79.5%, respectively. St Jude's classification stage IV disease was associated with poor survival (hazard ratio [HR] = 13.37, P = 0.037). Two patients (12.5%) had chronic rejection after the treatment for PTLD and one patient (6.3%) developed graft failure.
Conclusion:
PTLD is a major complication in pediatric LT recipients, but long-term survival is possible in most cases with an adequate treatment strategy. Stage IV disease is a major risk factor for poor survival in pediatric PTLD patients. During the management of PTLD, the possibility of chronic rejection and even graft failure should be considered.
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