Department of Hematology and INSERM U1245, Centre Henri Becquerel, Rouen, France.
Department of Hematology, University Hospital, Dijon, France.
Blood Adv. 2021 Oct 12;5(19):3862-3872. doi: 10.1182/bloodadvances.2021004778.
Primary mediastinal B-cell lymphoma (PMBL) is a rare type of aggressive lymphoma typically affecting young female patients. The first-line standard of care remains debated. We performed a large multicenter retrospective study in 25 centers in France and Belgium to describe PMBL patient outcomes after first-line treatment in real-life settings. A total of 313 patients were enrolled and received rituximab (R) plus ACVBP (doxorubicin, cyclophosphamide, vindesine, bleomycin, and prednisone) (n = 180) or CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) delivered every 14 days (R-CHOP14, n = 76) or 21 days (R-CHOP21, n = 57) and consolidation strategies in modalities that varied according to time and institution, mainly guided by positron emission tomography. Consolidation autologous stem cell transplantation was performed for 46 (25.6%), 24 (31.6%), and 1 (1.8%) patient in the R-ACVBP, R-CHOP14, and R-CHOP21 groups, respectively (P < .001); only 17 (5.4%) patients received mediastinal radiotherapy. The end-of-treatment complete metabolic response rates were 86.3%, 86.8%, and 76.6% (P = .23) in the R-ACVBP, R-CHOP14, and R-CHOP21 groups. The median follow-up was 44 months, and the R-ACVBP, R-CHOP14, and R-CHOP21 three-year progression-free survival probabilities were 89.4% (95% confidence interval [CI], 84.8-94.2), 89.4% (95% CI, 82.7-96.6), and 74.7% (95% CI, 64-87.1) (P = .018). A baseline total metabolic tumor volume (TMTV) ≥360 cm3 was associated with a lower progression-free survival (hazard ratio, 2.18; 95% CI, 1.05-4.53). Excess febrile neutropenia (24.4% vs 5.3% vs 5.3%; P < .001) and mucositis (22.8% vs 3.9% vs 1.8%; P < .001) were observed with R-ACVBP compared with the R-CHOP regimens. Patients with PMBL treated with dose-dense immunochemotherapy without radiotherapy have excellent outcomes. R-ACVBP acute toxicity was higher than that of R-CHOP14. Our data confirmed the prognostic importance of baseline TMTV.
原发性纵隔 B 细胞淋巴瘤（PMBL）是一种罕见的侵袭性淋巴瘤，主要影响年轻女性患者。一线标准治疗方案仍存在争议。我们在法国和比利时的 25 个中心进行了一项大型多中心回顾性研究，以描述 PMBL 患者在真实环境下一线治疗后的结局。共纳入 313 例患者，接受利妥昔单抗（R）联合 ACVBP（多柔比星、环磷酰胺、长春新碱、博来霉素和泼尼松）（n = 180）或 CHOP（环磷酰胺、多柔比星、长春新碱和泼尼松）每 14 天（R-CHOP14，n = 76）或 21 天（R-CHOP21，n = 57），并根据时间和机构采用不同的巩固策略，主要根据正电子发射断层扫描进行指导。46 例（25.6%）、24 例（31.6%）和 1 例（1.8%）患者分别在 R-ACVBP、R-CHOP14 和 R-CHOP21 组中接受了自体干细胞移植巩固治疗（P <.001）；只有 17 例（5.4%）患者接受纵隔放疗。R-ACVBP、R-CHOP14 和 R-CHOP21 组的治疗结束时完全代谢缓解率分别为 86.3%、86.8%和 76.6%（P =.23）。中位随访时间为 44 个月，R-ACVBP、R-CHOP14 和 R-CHOP21 组的 3 年无进展生存率分别为 89.4%（95%CI，84.8-94.2）、89.4%（95%CI，82.7-96.6）和 74.7%（95%CI，64-87.1）（P =.018）。基线时总代谢肿瘤体积（TMTV）≥360 cm3 与无进展生存率降低相关（风险比，2.18；95%CI，1.05-4.53）。与 R-CHOP 方案相比，R-ACVBP 组的发热性中性粒细胞减少症（24.4%比 5.3%比 5.3%；P <.001）和粘膜炎（22.8%比 3.9%比 1.8%；P <.001）发生率更高。未接受放疗的 PMBL 患者接受密集剂量免疫化疗后有良好的疗效。R-ACVBP 的急性毒性高于 R-CHOP14。我们的数据证实了基线 TMTV 的预后重要性。
