Istituto di Ematologia, Università Cattolica Sacro Cuore, Divisione di Ematologia, Policlinico Universitario Agostino Gemelli.
Mediterr J Hematol Infect Dis. 2009 Dec 14;1(2):e2009019. doi: 10.4084/MJHID.2009.019.
Epstein-Barr virus related lymphoproliferative disorders are a rare but potentially fatal complication of allogeneic stem cell transplantation with an incidence of 1-3% and occurring within 6 months after transplantation. The most relevant risk factors include the use of in vivo T-cell depletion with antithymocyte globulin, HLA disparities between donor and recipient, donor type, splenectomy etc. The higher the numbers of risk factors the higher the risk of developing Epstein-Barr virus related lymphoproliferative disorders. Monitoring EBV viremia after transplantation is of value and it should be applied to high risk patients since it allows pre-emptive therapy initiation at specified threshold values and early treatment. This strategy might reduce mortality which was >80% prior to the implementation of anti-EBV therapy. Treatment of EBV-LPD after allogeneic SCT may consist of anti-B-cell therapy (rituximab), adoptive T-cell immunotherapy or both. Rituximab treatment should be considered the first treatment option, preferably guided by intensive monitoring of EBV DNA while reduction of immunosuppression should be carefully evaluated for the risk of graft versus host disease.
EB 病毒相关淋巴组织增生性疾病是异基因造血干细胞移植的一种罕见但潜在致命的并发症，发生率为 1-3%，发生在移植后 6 个月内。最相关的危险因素包括使用抗胸腺细胞球蛋白进行体内 T 细胞耗竭、供者和受者之间 HLA 不匹配、供者类型、脾切除术等。危险因素越多，发生 EB 病毒相关淋巴组织增生性疾病的风险越高。移植后监测 EBV 病毒血症具有重要价值，应在高危患者中应用，因为它可以在特定的阈值下进行抢先治疗，并进行早期治疗。这一策略可能降低死亡率，在实施抗 EBV 治疗之前，死亡率>80%。异基因 SCT 后 EBV-LPD 的治疗可能包括抗 B 细胞治疗（利妥昔单抗）、过继性 T 细胞免疫治疗或两者兼有。利妥昔单抗治疗应被视为首选治疗方案，最好在密集监测 EBV DNA 的同时进行，而减少免疫抑制应仔细评估移植物抗宿主病的风险。
