Department of Lymphoid Malignancies, Maria Sklodowska-Curie National Research Institute of Oncology, 5 WK Roentgen Str, 02-781, Warsaw, Poland.
Department of Pathology and Laboratory Diagnostics, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland.
Sci Rep. 2022 Jun 22;12(1):10551. doi: 10.1038/s41598-022-14067-3.
Primary mediastinal B-cell lymphoma (PMBL) is currently curable in 85-95% of patients. Treatment regimens frequently used include RCHOP ± radiotherapy, DAEPOCH-R, or occasionally more intensive protocols. Here we present results of treatment of 124 patients with PMBL over a period between 2004 and 2017 with the use of a protocol designed for aggressive B-cell lymphoma GMALL/B-ALL/NHL2002 including 6 cycles of alternating immunochemotherapy with intermediate-dose methotrexate in each cycle, and reduced total doxorubicin dose (100 mg/m for whole treatment). Majority of patients (77%) received consolidative radiotherapy. A median (range) age of patients was 30 (18-59) years, and 60% were female. With a median (range) follow up of 9 (1-17) years, 5-year overall survival (OS) and 5-year progression free survival (PFS) were 94% and 92%, respectively. Positron emission tomography-computed tomography (PET-CT) results at the end of chemotherapy were predictive for outcome: OS and PFS at 5 year were 96% and 94% in PET-CT negative patients, respectively, and 70% and 70% in PET-CT-positive patients (p = 0.004 for OS, p = 0.01 for PFS). Eight (6%) patients had recurrent/refractory disease, however, no central nervous system (CNS) relapse was observed. Acute toxicity included pancytopenia grade 3/4, neutropenic fever, and treatment related mortality rate of 0.8%. Second malignancies and late cardiotoxicity occurred in 2.4% and 2.4% of patients, respectively. Intensive alternating immunochemotherapy protocol GMALL/B-ALL/NHL2002 is curative for more than 90% of PMBL patients and late toxicity in young patients is moderated. The attenuated dose of doxorubicin and intermediate dose of methotrexate may contribute to low incidence of late cardiotoxicity and effective CNS prophylaxis.
原发性纵隔 B 细胞淋巴瘤 (PMBL) 目前在 85-95%的患者中可治愈。常用的治疗方案包括 RCHOP±放疗、DAEPOCH-R,或偶尔采用更强化疗方案。本文报告了 2004 年至 2017 年期间使用 GMALL/B-ALL/NHL2002 侵袭性 B 细胞淋巴瘤方案治疗 124 例 PMBL 患者的结果,该方案包括 6 个周期的交替免疫化疗,每个周期中包含中等剂量甲氨蝶呤,且阿霉素总剂量减少(整个治疗过程中 100mg/m)。大多数患者(77%)接受了巩固性放疗。患者中位(范围)年龄为 30(18-59)岁,60%为女性。中位(范围)随访时间为 9(1-17)年,5 年总生存率(OS)和 5 年无进展生存率(PFS)分别为 94%和 92%。化疗结束时的正电子发射断层扫描-计算机断层扫描(PET-CT)结果可预测预后:PET-CT 阴性患者的 5 年 OS 和 PFS 分别为 96%和 94%,PET-CT 阳性患者分别为 70%和 70%(OS 为 p=0.004,PFS 为 p=0.01)。8(6%)例患者出现复发/难治性疾病,但未观察到中枢神经系统(CNS)复发。急性毒性包括 3/4 级全血细胞减少、中性粒细胞减少性发热和治疗相关死亡率为 0.8%。2.4%和 2.4%的患者分别发生了第二恶性肿瘤和迟发性心脏毒性。GMALL/B-ALL/NHL2002 强化交替免疫化疗方案对超过 90%的 PMBL 患者具有治愈作用,且年轻患者的晚期毒性适中。阿霉素的剂量减少和中等剂量的甲氨蝶呤可能有助于降低迟发性心脏毒性和有效的 CNS 预防的发生率。