背景:移植后淋巴增生性疾病(PTLD)是在实体器官或异基因造血干细胞移植(allo-HSCT)后发生的罕见但严重的并发症,进展迅速,死亡率高。原发性中枢神经系统(CNS)-PTLD在组织学上很少被识别。此外,EB病毒(EBV)DNA拷贝在CNS-PTLD中的诊断价值尚不清楚.
目的:我们在此报告一例单形EBV相关CNS-PTLD(弥漫性大B细胞淋巴瘤,DLBCL)后进行allo-HSCT,并进行荟萃分析以评估近年来PTLD治疗策略的疗效。
方法:我们提供的病例报告涵盖临床表现,诊断,治疗,和原发性CNS-PTLD患者的结局。此外,我们对431例PTLD患者allo-HSCT后的临床特征进行了系统评价和荟萃分析.我们评估PTLD管理的主要治疗方案和结果,包括利妥昔单抗,化疗,和自体或人类白细胞抗原(HLA)匹配的EBV特异性细胞毒性T淋巴细胞输注(EBV-CTL)/供体淋巴细胞输注(DLI)。
结果:荟萃分析显示,单独使用利妥昔单抗的总缓解率为69.0%(95%CI:0.47-0.84),利妥昔单抗加化疗为45.0%(95%CI:0.15-0.80),利妥昔单抗加EBV-CTLs/DLI的比例为91.0%(95%CI:0.83-0.96)。PTLD治疗后完全缓解(CR)率为67.0%(95%CI:0.56-0.77)。此外,6个月和1年总生存率(OS)分别为64.0%(95%CI:0.31-0.87)和49.0%(95%CI:0.31-0.68),分别。
结论:此案例强调了迫切需要有效,CNS-PTLD的低毒性治疗方案。我们的荟萃分析表明,利妥昔单抗联合EBV-CTLs/DLI可能是allo-HSCT后PTLD管理的有利策略。
BACKGROUND: Post-transplant lymphoproliferative disorders (PTLD) are rare but severe complications that occur after solid organ or allogeneic hematopoietic stem cell transplantations (allo-HSCT), with rapid progression and high mortality. Primary central nervous system (CNS)-PTLD are rarely recognized histo-pathologically. In addition, the diagnostic value of the Epstein-Barr virus (EBV) DNA copies in CNS-PTLD remains poorly understood.
OBJECTIVE: We herein report a
case of monomorphic EBV-associated CNS-PTLD (diffuse large B-cell lymphoma, DLBCL) after allo-HSCT and perform a meta-analysis to assess the efficacy of PTLD treatment strategies in recent years.
METHODS: We present the
case report covering clinical manifestations, diagnosis, treatment, and outcomes of a patient with primary CNS-PTLD. Additionally, we include a systematic review and meta-analysis of the clinical characteristics of 431 patients with PTLD after allo-HSCT. We evaluate the main treatment options and outcomes of PTLD management, including rituximab, chemotherapies, and autologous or human leukocyte antigen (HLA)-matched EBV-specific cytotoxic T lymphocyte infusion (EBV-CTLs)/donor lymphocyte infusion (DLI).
RESULTS: The meta-analysis revealed an overall response rate of 69.0% for rituximab alone (95% CI: 0.47-0.84), 45.0% for rituximab plus chemotherapies (95% CI: 0.15-0.80), and 91.0% for rituximab plus EBV-CTLs/DLI (95% CI: 0.83-0.96). The complete response (CR) rate after treatments for PTLD was 67.0% (95% CI: 0.56-0.77). Moreover, the 6-month and 1-year overall survival (OS) rate was 64.0% (95% CI: 0.31-0.87) and 49.0% (95% CI: 0.31-0.68), respectively.
CONCLUSIONS: This
case highlighted the urgent need for effective, low-toxic treatment regimens for CNS-PTLD. Our meta-analysis suggested that rituximab combined with EBV-CTLs/DLI could be a favorable strategy for the management of PTLD after allo-HSCT.