Internal Medicine Residency Training Program, School of Medicine, University of Colorado, 12631 East 17th Avenue, B177 Academic Office 1, Aurora, CO, 80045, USA.
Division of Hematology, School of Medicine, University of Colorado, 1665 Aurora Court, MS F754, Aurora, CO, 80045, USA.
Curr Treat Options Oncol. 2018 May 24;19(7):33. doi: 10.1007/s11864-018-0549-6.
Post-transplant lymphoproliferative disorder (PTLD) is one of the most common neoplasms seen after solid organ and hematopoietic stem cell transplantation, and is associated with significant morbidity and mortality. The pathogenesis is related to post-transplant immunosuppression and EBV infection. Prevention of PTLD depends upon judicious use of immunosuppression and serial EBV monitoring. Preemptive therapy consists of reduction of immunosuppression, antiviral medications, and single-agent rituximab. There are no randomized phase III trials on PTLD treatment, so current management guidelines are largely based on recent phase II trials, single-institution retrospective studies, and expert opinion. Management of PTLD is dependent upon its subtypes. Early-type and polymorphic PTLD generally respond to reduction of immunosuppression and rituximab monotherapy, whereas monomorphic PTLD often requires additional concurrent or sequential use of chemotherapy. For rare subtypes of PTLD, standard-of-care guidelines for de novo lymphomas are recommended. Surgical resection or radiotherapy may be used as adjunctive therapy depending on the extent of disease. Non-chemotherapy options such as adoptive T cell therapy have shown promising efficacy and must be explored further. Despite progress in the last decade, overall survival rates continue to be low in published series. This review highlights the need for prospective randomized trials incorporating novel agents to improve outcomes in PTLD.
移植后淋巴组织增生性疾病（PTLD）是实体器官和造血干细胞移植后最常见的肿瘤之一，与显著的发病率和死亡率相关。其发病机制与移植后免疫抑制和 EBV 感染有关。PTLD 的预防取决于免疫抑制药物的合理使用和 EBV 的连续监测。抢先治疗包括减少免疫抑制、抗病毒药物和单克隆抗体利妥昔单抗。目前尚无关于 PTLD 治疗的随机 III 期临床试验，因此当前的管理指南主要基于最近的 II 期试验、单机构回顾性研究和专家意见。PTLD 的管理取决于其亚型。早期和多形性 PTLD 通常对减少免疫抑制和利妥昔单抗单药治疗有反应，而单形性 PTLD 通常需要额外的联合或序贯化疗。对于罕见的 PTLD 亚型，建议采用新诊断淋巴瘤的标准治疗指南。根据疾病的范围，可以使用手术切除或放射治疗作为辅助治疗。非化疗选择，如过继性 T 细胞治疗，已显示出有希望的疗效，需要进一步探索。尽管在过去十年中取得了进展，但在已发表的系列中，总体生存率仍然较低。本综述强调了需要进行前瞻性随机试验，纳入新的药物，以改善 PTLD 的治疗效果。
