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Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Transplant. Feb 28, 2020; 10(2): 29-46
Published online Feb 28, 2020. doi: 10.5500/wjt.v10.i2.29
Post-transplantation lymphoproliferative disorders: Current concepts and future therapeutic approaches
Fedaey Abbas, Mohsen El Kossi, Ihab Sakr Shaheen, Ajay Sharma, Ahmed Halawa
Fedaey Abbas, Nephrology Department, Jaber El Ahmed Military Hospital, Safat 13005, Kuwait
Fedaey Abbas, Mohsen El Kossi, Ihab Sakr Shaheen, Ajay Sharma, Ahmed Halawa, Faculty of Health and Science, University of Liverpool, Institute of Learning and Teaching, School of Medicine, Liverpool L69 3GB, United Kingdom
Mohsen El Kossi, Doncaster Royal Infirmary, Doncaster DN2 5LT, United Kingdom
Ihab Sakr Shaheen, Department of Paediatric Nephrology, Royal Hospital for Children, Glasgow G51 4TF, United Kingdom
Ajay Sharma, Department of Transplant Surgery, Royal Liverpool University Hospitals, Liverpool L7 8XP, United Kingdom
Ahmed Halawa, Department of Transplantation, Sheffield Teaching Hospitals, Sheffield S57AU, United Kingdom
Author contributions: Abbas F performed research; designed the study and wrote the paper. El Kossi M, Shaheen IS and Sharma A analyzed data, contributed new reagents and analytic tools, reviewed and edited the manuscript. Halawa A conceptualized and designed the study, supervised the data collection, and reviewed and edited the manuscript.
Conflict-of-interest statement: No conflict of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Ahmed Halawa, FRSC, MD, Associate Professor, Department of Transplantation, Sheffield Teaching Hospital, Herries Road, Sheffield S5 7AU, United Kingdom. ahmed.halawa@liverpool.ac.uk
Received: September 17, 2019
Peer-review started: September 17, 2019
First decision: October 14, 2019
Revised: October 21, 2019
Accepted: December 13, 2019
Article in press: December 13, 2019
Published online: February 28, 2020
Processing time: 163 Days and 19.5 Hours
Abstract

Transplant recipients are vulnerable to a higher risk of malignancy after solid organ transplantation and allogeneic hematopoietic stem-cell transplant. Post-transplant lymphoproliferative disorders (PTLD) include a wide spectrum of diseases ranging from benign proliferation of lymphoid tissues to frank malignancy with aggressive behavior. Two main risk factors of PTLD are: Firstly, the cumulative immunosuppressive burden, and secondly, the oncogenic impact of the Epstein-Barr virus. The latter is a key pathognomonic driver of PTLD evolution. Over the last two decades, a considerable progress has been made in diagnosis and therapy of PTLD. The treatment of PTLD includes reduction of immunosuppression, rituximab therapy, either isolated or in combination with other chemotherapeutic agents, adoptive therapy, surgical intervention, antiviral therapy and radiotherapy. In this review we shall discuss the prevalence, clinical clues, prophylactic measures as well as the current and future therapeutic strategies of this devastating disorder.

Keywords: Lymphoproliferative disorders; Epstein-Barr virus; Solid organ transplant; Hematopoietic stem cell transplant; Post-transplant lymphoproliferative disorder prevention; Future therapies

Core tip: Post-transplant lymphoproliferative disorders (PTLD) is a serious complication related to the intensity of post-transplant immunosuppression. The role of Epstein-Barr virus (EBV) in PTLD evolution is well established; however, development of PTLD in EBV negative patients is not uncommon. The key step in the management of PTLD is to reduce the immunosuppressive load. Transplant clinicians should be vigilant to the possibility of this complication, particularly in patients with past history of exposure to immunosuppression during treatment of the primary renal disease. High index of suspicion is crucial for timely diagnosis. Therapeutic options include rituximab, chemotherapy, antivirals, adoptive therapy and surgery.