移植后淋巴增生性疾病（PTLD）是罕见和异质性的淋巴增生，由于实体器官移植（SOT）和造血干细胞移植（HSCT）后的免疫抑制而发生，其中大部分由EBV驱动。尽管某些组织学类似于免疫能力患者中观察到的淋巴肿瘤，但由于病理生物学差异和治疗并发症风险较高，PTLD的治疗可能不同。在未能减少免疫抑制（RIS）的SOT PTLD中，最常见的治疗方法考虑以基于第2期研究的利妥昔单抗+/-化疗的风险分层序列算法。在HSCT PTLD中，仅RIS和化疗通常无效，利妥昔单抗+/- RIS成为一线治疗的黄金标准。在本篇综述中，我们将介绍RIS以外的PTLD治疗更新情况。我们强调了最近尝试将更激进的化疗方案和新型治疗纳入传统的风险分层序列方法的研究。我们还探讨了EBV-细胞毒素T淋巴细胞在治疗EBV驱动的PTLD中的作用。此外，我们还将讨论其他有潜力在PTLD中发挥作用的新型药物，以及在这个人群中采用嵌合抗原受体T细胞疗法和免疫检查点抑制剂可能会带来的挑战。 ©2023 The Authors. British Journal of Haematology由英国血液学会和John Wiley＆Sons Ltd.出版

Post-transplant lymphoproliferative disorder (PTLD) is rare and heterogeneous lymphoid proliferations that occur as a result of immunosuppression following solid organ transplant (SOT) and haematopoietic stem cell transplant (HSCT) with the majority being driven by EBV. Although some histologies are similar to lymphoid neoplasms seen in immunocompetent patients, treatment of PTLD may be different due to difference in pathobiology and higher risk of treatment complications. The most common treatment approach in SOT PTLD after failing immunosuppression reduction (RIS) takes into consideration a risk-stratified sequential algorithm with rituximab +/- chemotherapy based on phase 2 studies. In HSCT PTLD, RIS alone and chemotherapy are usually ineffective making rituximab +/- RIS as the gold standard of frontline treatment. In this review, we give an update on the treatment of PTLD beyond RIS. We highlight the most recent studies that attempted to incorporate more aggressive chemotherapy regimens and novel treatments into the traditional risk-stratified sequential approach. We also discuss the role of EBV-cytotoxic T lymphocytes in treatment of EBV-driven PTLD. Other novel agents with potential role in PTLD will be discussed in addition to the challenges that could arise with chimeric antigen receptor T-cell therapy and immune checkpoint inhibitors in this population.© 2023 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.