Fulchiero Rosanna, Amaral Sandra
Department of Nephrology, Children's Hospital of Philadelphia, Philadelphia, PA, United States.
Department of Biostatistics, Epidemiology and Informatics, University of Pennsylvania, Philadelphia, PA, United States.
Front Pediatr. 2022 Dec 7;10:1087864. doi: 10.3389/fped.2022.1087864. eCollection 2022.
Post-transplant lymphoproliferative disease (PTLD) is the most common malignancy complicating solid organ transplantation (SOT) in adults and children. PTLD encompasses a spectrum of histopathologic features and organ involvement, ranging from benign lymphoproliferation and infectious-mononucleosis like presentation to invasive neoplastic processes such as classical Hodgkin lymphoma. The predominant risk factors for PTLD are Epstein-Barr virus (EBV) serostatus at the time of transplant and the intensity of immunosuppression following transplantation; with EBV-negative recipients of EBV-positive donor organs at the highest risk. In children, PTLD commonly presents in the first two years after transplant, with 80% of cases in the first year, and over 90% of cases associated with EBV-positive B-cell proliferation. Though pediatric kidney transplant recipients are at lower risk (1-3%) for PTLD compared to their other SOT counterparts, there is still a significant risk of morbidity, allograft failure, and an estimated 5-year mortality rate of up to 50%. In spite of this, there is no consensus for monitoring of at-risk patients or optimal management strategies for pediatric patients with PTLD. Here we review pathogenesis and risk factors for the development of PTLD, with current practices for prevention, diagnosis, and management of PTLD in pediatric kidney transplant recipients. We also highlight emerging concepts, current research gaps and potential future developments to improve clinical outcomes and longevity in these patients.
移植后淋巴细胞增生性疾病（PTLD）是成人和儿童实体器官移植（SOT）后最常见的并发症。PTLD涵盖一系列组织病理学特征和器官受累情况，从良性淋巴细胞增生和类似传染性单核细胞增多症的表现到侵袭性肿瘤性病变，如经典霍奇金淋巴瘤。PTLD的主要危险因素为移植时的爱泼斯坦-巴尔病毒（EBV）血清学状态以及移植后免疫抑制的强度；接受EBV阳性供体器官的EBV阴性受者风险最高。在儿童中，PTLD通常在移植后的头两年出现，80%的病例在第一年出现，超过90%的病例与EBV阳性B细胞增殖有关。尽管与其他实体器官移植受者相比，小儿肾移植受者发生PTLD的风险较低（1%-3%），但仍存在显著的发病风险、移植肾失功风险，估计5年死亡率高达50%。尽管如此，对于PTLD高危患儿的监测或最佳管理策略尚无共识。在此，我们回顾PTLD发生的发病机制和危险因素，以及小儿肾移植受者PTLD预防、诊断和管理的现行做法。我们还强调了新出现的概念、当前的研究空白以及未来可能的发展方向，以改善这些患者的临床结局和生存期。
