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Post-transplant lymphoproliferative disorder in children: incidence, prognosis, and treatment options
1Service d'Hémato-immunologie, Hôpital Robert Debré, Paris, France. albert.faye@rdb.ap-hop-paris.fr
Insights
Post-transplant lymphoproliferative disorder (PTLD) is a serious complication in children following transplantation. New strategies and treatments show promise for controlling PTLD and improving patient outcomes.
Area of Science:
- Pediatric Hematology/Oncology
- Transplantation Immunology
- Infectious Diseases
Background:
- Post-transplant lymphoproliferative disorder (PTLD) is a significant cause of mortality in pediatric solid organ and hematopoietic stem cell transplant recipients.
- PTLDs are heterogeneous B-cell proliferations often linked to Epstein-Barr virus (EBV) reactivation under immunosuppression.
- Key risk factors include EBV serostatus mismatch, immunosuppression intensity, T-cell depletion, and HLA mismatch.
Purpose of the Study:
- To review current understanding and management of PTLD in pediatric transplant recipients.
- To highlight evolving preventive and pre-emptive strategies for PTLD control.
- To discuss novel therapeutic approaches and their potential impact on PTLD outcomes.
Main Methods:
- Literature review of PTLD pathophysiology, risk factors, and treatment modalities.
- Analysis of traditional and emerging therapeutic strategies for PTLD.
- Discussion of the role of EBV and immunosuppression in PTLD development.
Main Results:
- Historically, immunosuppression reduction was the primary PTLD treatment, risking graft rejection.
- Emerging preventive and pre-emptive strategies aim to mitigate PTLD risk.
- Novel therapies including anti-B-cell antibodies and anti-EBV T-cells show therapeutic promise.
Conclusions:
- PTLD remains a critical challenge in pediatric transplantation, necessitating advanced management strategies.
- Preventive and pre-emptive approaches, guided by predictive markers, are crucial for PTLD control.
- Further evaluation of novel therapies, particularly in pre-emptive contexts, is essential to improve PTLD outcomes.
Abstract:
Post-transplant lymphoproliferative disorder (PTLD) after solid organ or hematopoietic stem cell transplantation in children is a serious complication that has been responsible for high mortality rates over recent years. PTLDs are part of a clinically and histologically heterogeneous group of B-lymphocyte proliferations mostly induced by Epstein-Barr virus (EBV) in a context of immunosuppression. Major risk factors for PTLDs in solid organ transplantation are the EBV serostatus mismatch and the intensity, duration, and type of immunosuppression. T-cell depletion and the HLA-mismatched donor and recipient are the main risk factors following hematopoietic stem cell transplantation. For a long time, the only safe and effective therapeutic approach to PTLD was reduction of immunosuppression, with a risk of graft rejection. Based on a better knowledge of the pathophysiology and risk factors for PTLD, preventive and pre-emptive strategies have been recently proposed to control PTLD. New treatment modalities, such as anti-B-cell antibodies, cytokine inhibitor therapy, or anti-EBV cytotoxic T lymphocytes are promising and may improve the outcome of PTLD. These therapeutic approaches need to be further evaluated, especially in the context of pre-emptive strategies adapted to predictive markers of EBV-induced PTLD.
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