Styczynski Jan, van der Velden Walter, Fox Christopher P, Engelhard Dan, de la Camara Rafael, Cordonnier Catherine, Ljungman Per
Department of Pediatric Hematology and Oncology, Collegium Medicum, Nicolaus Copernicus University Torun, Jurasz University Hospital, Bydgoszcz, Poland
Department of Hematology, Radboud University Medical Centre, Nijmegen, The Netherlands.
Haematologica. 2016 Jul;101(7):803-11. doi: 10.3324/haematol.2016.144428.
Epstein-Barr virus-related post-transplant lymphoproliferative disorders are recognized as a significant cause of morbidity and mortality in patients undergoing hematopoietic stem cell transplantation. To better define current understanding of post-transplant lymphoproliferative disorders in stem cell transplant patients, and to improve its diagnosis and management, a working group of the Sixth European Conference on Infections in Leukemia 2015 reviewed the literature, graded the available quality of evidence, and developed evidence-based recommendations for diagnosis, prevention, prophylaxis and therapy of post-transplant lymphoproliferative disorders exclusively in the stem cell transplant setting. The key elements in diagnosis include non-invasive and invasive methods. The former are based on quantitative viral load measurement and imaging with positron emission tomography; the latter with tissue biopsy for histopathology and detection of Epstein-Barr virus. The diagnosis of post-transplant lymphoproliferative disorder can be established on a proven or probable level. Therapeutic strategies include prophylaxis, preemptive therapy and targeted therapy. Rituximab, reduction of immunosuppression and Epstein-Barr virus-specific cytotoxic T-cell therapy are recommended as first-line therapy, whilst unselected donor lymphocyte infusions or chemotherapy are options as second-line therapy; other methods including antiviral drugs are discouraged.
爱泼斯坦-巴尔病毒相关的移植后淋巴细胞增生性疾病被认为是接受造血干细胞移植患者发病和死亡的重要原因。为了更好地明确目前对干细胞移植患者移植后淋巴细胞增生性疾病的认识，并改善其诊断和管理，2015年第六届欧洲白血病感染会议的一个工作组回顾了文献，对现有证据质量进行分级，并专门针对干细胞移植环境下移植后淋巴细胞增生性疾病的诊断、预防、 prophylaxis和治疗制定了基于证据的建议。诊断的关键要素包括非侵入性和侵入性方法。前者基于定量病毒载量测量和正电子发射断层扫描成像；后者通过组织活检进行组织病理学检查和爱泼斯坦-巴尔病毒检测。移植后淋巴细胞增生性疾病的诊断可以在确诊或疑似水平上确立。治疗策略包括预防、抢先治疗和靶向治疗。推荐利妥昔单抗、降低免疫抑制和爱泼斯坦-巴尔病毒特异性细胞毒性T细胞治疗作为一线治疗，而未选择的供体淋巴细胞输注或化疗作为二线治疗选择；不鼓励使用包括抗病毒药物在内的其他方法。
