Department of Oncology - Haematology Unit, AOU Policlinico P. Giaccone, Palermo, Italy.
Fondazione Italiana Linfomi (FIL) c/o Department of Diagnostic, Clinical and Public Health Medicine, University of Modena and Reggio Emilia, Modena, Italy.
Br J Haematol. 2018 Dec;183(5):755-765. doi: 10.1111/bjh.15641. Epub 2018 Nov 8.
Splenectomy in addition to immunotherapy with rituximab can provide quick and sometimes durable disease control in patients with splenic marginal zone lymphoma (SMZL). However, systemic chemotherapy is ultimately required in many cases. The BRISMA (Bendamustine-rituximab as first-line treatment of splenic marginal zone lymphoma)/IELSG (International Extranodal Lymphoma Study Group)36 trial is an open-label, single arm phase II study designed by the IELSG in cooperation with the Fondazione Italiana Linfomi and the lymphoma Study Association according to Simon's two-stage method. The primary endpoint was complete response rate. Fifty-six patients with SMZL diagnosis confirmed on central revision were treated with bendamustine (90 mg/m days 1, 2) and rituximab (375 mg/m day 1) every 28 days for six cycles (B-R). The overall response and CR rates were 91% and 73%, respectively. Duration of response, progression-free survival and overall survival at 3 years were 93% (95% confidence interval [CI] 81-98), 90% (95% CI 77-96) and 96% (95% CI 84-98), respectively. Toxicity was mostly haematological. Neutropenia grade ≥3 was recorded in 43% of patients; infections and febrile neutropenia in 5·4% and 3·6%. Overall, 14 patients (25%) experienced serious adverse events. Five patients (9%) went off-study because of toxicity and one patient died from infection. In conclusion, B-R resulted in a very effective first-line regimen for SMZL. Based on the results achieved in the BRISMA trial, B-R should be considered when a chemotherapy combination with rituximab is deemed necessary for symptomatic SMZL patients.
脾切除术联合利妥昔单抗免疫治疗可在脾边缘区淋巴瘤（SMZL）患者中迅速且有时持久地控制疾病。然而，许多情况下最终仍需要全身化疗。BRISMA（苯达莫司汀-利妥昔单抗作为脾边缘区淋巴瘤的一线治疗）/IELSG（国际结外淋巴瘤研究组）36 试验是由 IELSG 与意大利淋巴瘤基金会和淋巴瘤研究协会合作根据 Simon 的两阶段法设计的一项开放性、单臂、二期研究。主要终点为完全缓解率。56 例经中心修订后确诊的 SMZL 患者接受苯达莫司汀（90mg/m ，第 1、2 天）和利妥昔单抗（375mg/m ，第 1 天）治疗，每 28 天为一个周期，共 6 个周期（B-R）。总体缓解率和完全缓解率分别为 91%和 73%。3 年时的缓解持续时间、无进展生存期和总生存期分别为 93%（95%可信区间 [CI] 81-98）、90%（95% CI 77-96）和 96%（95% CI 84-98）。毒性主要为血液学毒性。43%的患者发生≥3 级中性粒细胞减少症；5.4%和 3.6%的患者分别发生感染和发热性中性粒细胞减少症。总体而言，14 名患者（25%）发生严重不良事件。5 名患者（9%）因毒性而退出研究，1 名患者因感染而死亡。总之，B-R 是治疗 SMZL 的一种非常有效的一线方案。基于 BRISMA 试验的结果，对于有症状的 SMZL 患者，在考虑使用含利妥昔单抗的化疗方案时，应考虑 B-R。
