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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Format: | Article |
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Elsevier
2019-11-01
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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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institution | Directory Open Access Journal |
issn | 0929-6646 |
language | English |
last_indexed | 2024-12-12T17:43:32Z |
publishDate | 2019-11-01 |
publisher | Elsevier |
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series | Journal of the Formosan Medical Association |
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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