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实体器官移植术后儿童患者移植后淋巴细胞增生性疾病的诊断、预防和治疗现状:ERN移植儿童医疗工作组调查结果

Current Practices on Diagnosis, Prevention and Treatment of Post-Transplant Lymphoproliferative Disorder in Pediatric Patients after Solid Organ Transplantation: Results of ERN TransplantChild Healthcare Working Group Survey.

作者信息

Baker Alastair, Frauca Remacha Esteban, Torres Canizales Juan, Bravo-Gallego Luz Yadira, Fitzpatrick Emer, Alonso Melgar Angel, Muñoz Bartolo Gema, Garcia Guereta Luis, Ramos Boluda Esther, Mozo Yasmina, Broniszczak Dorota, Jarmużek Wioletta, Kalicinski Piotr, Maecker-Kolhoff Britta, Carlens Julia, Baumann Ulrich, Roy Charlotte, Chardot Christophe, Benetti Elisa, Cananzi Mara, Calore Elisabetta, Dello Strologo Luca, Candusso Manila, Lopes Maria Francelina, Brito Manuel João, Gonçalves Cristina, Do Carmo Carmen, Stephenne Xavier, Wennberg Lars, Stone Rosário, Rascon Jelena, Lindemans Caroline, Turkiewicz Dominik, Giraldi Eugenia, Nicastro Emanuele, D'Antiga Lorenzo, Ackermann Oanez, Jara Vega Paloma

机构信息

Paediatric Liver, Gastrointestinal and Nutrition Centre, School of Medicine, King's College Hospital, King's College London, Denmark Hill, London SE5 9RS, UK.

Servicio de Hepatología Pediátrica, Hospital Universitario La Paz, 28046 Madrid, Spain.

出版信息

Children (Basel). 2021 Jul 29;8(8):661. doi: 10.3390/children8080661.

Abstract

(1) Background: Post-transplant lymphoproliferative disease (PTLD) is a significant complication of solid organ transplantation (SOT). However, there is lack of consensus in PTLD management. Our aim was to establish a present benchmark for comparison between international centers and between various organ transplant systems and modalities; (2) Methods: A cross-sectional questionnaire of relevant PTLD practices in pediatric transplantation was sent to multidisciplinary teams from 17 European center members of ERN TransplantChild to evaluate the centers' approach strategies for diagnosis and treatment and how current practices impact a cross-sectional series of PTLD cases; (3) Results: A total of 34 SOT programs from 13 European centers participated. The decision to start preemptive treatment and its guidance was based on both EBV viremia monitoring plus additional laboratory methods and clinical assessment (61%). Among treatment modalities the most common initial practice at diagnosis was to reduce the immunosuppression (61%). A total of 126 PTLD cases were reported during the period 2012-2016. According to their histopathological classification, monomorphic lesions were the most frequent (46%). Graft rejection after PTLD remission was 33%. Of the total cases diagnosed with PTLD, 88% survived; (4) Conclusions: There is still no consensus on prevention and treatment of PTLD, which implies the need to generate evidence. This might successively allow the development of clinical guidelines.

摘要

(1) 背景:移植后淋巴细胞增生性疾病(PTLD)是实体器官移植(SOT)的一种重要并发症。然而,PTLD的管理缺乏共识。我们的目的是建立一个当前的基准,用于国际中心之间以及各种器官移植系统和方式之间的比较;(2) 方法:向欧洲移植儿童网络(ERN TransplantChild)的17个欧洲中心成员的多学科团队发送了一份关于儿科移植中PTLD相关实践的横断面调查问卷,以评估各中心的诊断和治疗方法策略,以及当前实践如何影响一系列PTLD病例的横断面情况;(3) 结果:来自13个欧洲中心的34个SOT项目参与了调查。开始抢先治疗的决定及其指导基于EBV病毒血症监测以及其他实验室方法和临床评估(61%)。在治疗方式中,诊断时最常见的初始做法是降低免疫抑制(61%)。2012年至2016年期间共报告了126例PTLD病例。根据组织病理学分类,单形性病变最为常见(46%)。PTLD缓解后的移植物排斥率为33%。在诊断为PTLD的所有病例中,88%存活;(4) 结论:PTLD的预防和治疗仍未达成共识,这意味着需要生成证据。这可能会相继促成临床指南的制定。

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