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Post-transplant lymphoproliferative disorder following pediatric heart transplantation
Fernando Mendoza1, Hiroko Kunitake, Hillel Laks
1Division of Cardiothoracic Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095-1741, USA.
Insights
Post-transplant lymphoproliferative disorder (PTLD) affects 3.5% of pediatric heart transplant recipients. Key risk factors include Epstein-Barr virus (EBV) seronegativity and Rh incompatibility, impacting survival.
Area of Science:
- Pediatric Cardiology
- Transplantation Immunology
- Oncology
Background:
- Post-transplant lymphoproliferative disorder (PTLD) is a known complication of immunosuppression following organ transplantation.
- Pediatric heart transplant recipients have a higher prevalence of PTLD, yet data on incidence and risk factors remain limited.
Purpose of the Study:
- To investigate the incidence, risk factors, and outcomes of PTLD in pediatric cardiac allograft recipients.
- To identify specific patient and transplant-related factors associated with PTLD development in this population.
Main Methods:
- Retrospective analysis of medical records from 143 pediatric patients who received cardiac allografts between 1984 and 2002.
- Evaluation of patient demographics, transplant characteristics, and clinical outcomes, including PTLD diagnosis and survival.
Main Results:
- Five pediatric patients (3.5%) developed PTLD over a mean follow-up of 41.1 months.
- Significant risk factors identified by univariate analysis included Rh negativity (p=0.01), Rh mismatch (p=0.003), Epstein-Barr virus (EBV) seronegativity (p=0.001), and transplantation for congenital heart disease (p<0.02).
- PTLD was associated with significant morbidity and mortality, with a mean survival of 21.2 months post-diagnosis.
Conclusions:
- PTLD is a serious complication in pediatric heart transplantation, occurring in approximately 3.5% of recipients.
- EBV seronegativity and Rh negative status/mismatch are significant risk factors for PTLD in pediatric cardiac transplant patients, beyond general immunosuppression.
- Non-hematogenous malignancies are rare in this cohort.
Abstract:
Immunosuppression after heart transplantation is implicated in development of post-transplant lymphoproliferative disorder (PTLD). Despite a higher prevalence of PTLD in children, there is scarce knowledge about incidence, pathophysiologic mechanisms and risk factors for PTLD in pediatric recipients of cardiac allografts. We examined retrospectively the medical records of all 143 pediatric patients (mean age 9.2 +/- 6.1 yr) who received donor allografts between 1984 and 2002 and survived over 30 days. Five children (3.5%) developed PTLD over a mean follow-up period of 41.1 +/- 46.0 months. Time from transplant to diagnosis of PTLD ranged from 3.9 to 112 months (mean 48.0 +/- 41.9 months). Excluding PTLD, no other malignancies were found in this population. Actuarial freedom from PTLD was 99.2%, 99.2% and 96.2% at 1, 2, and 5 yr, respectively. Children who developed PTLD were more likely (by univariate analysis) to have been Rh negative (p = 0.01), Rh mismatched (p = 0.003), Epstein-Barr virus (EBV) seronegative (p = 0.001) and transplanted for congenital heart disease (p < 0.02). PTLD was associated with significant morbidity and mortality with a mean survival following diagnosis of 21.2 months. PTLD is a serious complicating outcome of cardiac transplantation that occurs in approximately 3.5% of children. Aside of immunosuppression, risk factors in this series for developing PTLD include EBV seronegativity and Rh negative status and mismatch. Non-hematogenous malignancies are rare in light of short allograft half-life.
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