Post-transplantation lymphoproliferative disease in pediatric liver recipients in Taiwan

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...

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Main Authors: Chien-Ting Hsu, Mei-Hwei Chang, Ming-Chih Ho, Hsiu-Hao Chang, Meng-Yao Lu, Shiann-Tarng Jou, Yen-Hsuan Ni, Huey-Ling Chen, Hong-Yuan Hsu, Jia-Feng Wu
Format: Article
Language:English
Published: Elsevier 2019-11-01
Series:Journal of the Formosan Medical Association
Online Access:http://www.sciencedirect.com/science/article/pii/S0929664618300093
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author Chien-Ting Hsu
Mei-Hwei Chang
Ming-Chih Ho
Hsiu-Hao Chang
Meng-Yao Lu
Shiann-Tarng Jou
Yen-Hsuan Ni
Huey-Ling Chen
Hong-Yuan Hsu
Jia-Feng Wu
author_facet Chien-Ting Hsu
Mei-Hwei Chang
Ming-Chih Ho
Hsiu-Hao Chang
Meng-Yao Lu
Shiann-Tarng Jou
Yen-Hsuan Ni
Huey-Ling Chen
Hong-Yuan Hsu
Jia-Feng Wu
author_sort Chien-Ting Hsu
collection DOAJ
description 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. Keywords: Cytomegalovirus, Epstein-Barr virus, Liver transplant, Lymphoma, Post-transplantation lympho- proliferative disease
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spelling doaj.art-d39e2f795c484b6b9b01b2aacb721bf52022-12-22T00:17:00ZengElsevierJournal of the Formosan Medical Association0929-66462019-11-011181115371545Post-transplantation lymphoproliferative disease in pediatric liver recipients in TaiwanChien-Ting Hsu0Mei-Hwei Chang1Ming-Chih Ho2Hsiu-Hao Chang3Meng-Yao Lu4Shiann-Tarng Jou5Yen-Hsuan Ni6Huey-Ling Chen7Hong-Yuan Hsu8Jia-Feng Wu9Department of Pediatrics, National Taiwan University Hospital, No. 8, Chung-Shan S. Rd., Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, No. 8, Chung-Shan S. Rd., Taipei, TaiwanDepartment of Surgery, National Taiwan University Hospital, No. 7, Chung-Shan S. Rd., Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, No. 8, Chung-Shan S. Rd., Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, No. 8, Chung-Shan S. Rd., Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, No. 8, Chung-Shan S. Rd., Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, No. 8, Chung-Shan S. Rd., Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, No. 8, Chung-Shan S. Rd., Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, No. 8, Chung-Shan S. Rd., Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital, No. 8, Chung-Shan S. Rd., Taipei, Taiwan; Corresponding author. Department of Pediatrics, National Taiwan University Hospital; No. 8, Chung-Shan S. Rd., Taipei, Taiwan. Fax: +886 2 23938871.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. Keywords: Cytomegalovirus, Epstein-Barr virus, Liver transplant, Lymphoma, Post-transplantation lympho- proliferative diseasehttp://www.sciencedirect.com/science/article/pii/S0929664618300093
spellingShingle Chien-Ting Hsu
Mei-Hwei Chang
Ming-Chih Ho
Hsiu-Hao Chang
Meng-Yao Lu
Shiann-Tarng Jou
Yen-Hsuan Ni
Huey-Ling Chen
Hong-Yuan Hsu
Jia-Feng Wu
Post-transplantation lymphoproliferative disease in pediatric liver recipients in Taiwan
Journal of the Formosan Medical Association
title Post-transplantation lymphoproliferative disease in pediatric liver recipients in Taiwan
title_full Post-transplantation lymphoproliferative disease in pediatric liver recipients in Taiwan
title_fullStr Post-transplantation lymphoproliferative disease in pediatric liver recipients in Taiwan
title_full_unstemmed Post-transplantation lymphoproliferative disease in pediatric liver recipients in Taiwan
title_short Post-transplantation lymphoproliferative disease in pediatric liver recipients in Taiwan
title_sort post transplantation lymphoproliferative disease in pediatric liver recipients in taiwan
url http://www.sciencedirect.com/science/article/pii/S0929664618300093
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