Abstract：
The aims of our study were to analyse compliance with the 2014 GELTAMO SMZL Guidelines, in patients with splenic marginal zone lymphoma (SMZL), and to evaluate the outcome according to the HPLLs/ABC-adapted therapeutic strategy. Observational prospective multicenter study of 181 SMZL patients diagnosed between 2014 and 2020. Lymphoma-specific survival (LSS), composite event-free survival (CEFS) and response rates were assessed. 57% of the 168 patients included in the analysis followed the Guidelines. The overall response rate was higher in the rituximab chemotherapy and in the rituximab arms compared with the splenectomy arm (p < 0.001). The 5-year overall survival was 77% and the 5-year LSS of 93%. There were no differences in the 5-year LSS according to the treatment received (p = 0.68). The 5-year CEFS in the overall series was 45%, and there were significant differences between scores A and B (p = 0.036). There were no significant differences when comparing LSS and progression-free survival in patients treated with rituximab or rituximab chemotherapy at diagnosis or after observation. Our data support HPLLs/ABC score as a practical tool for the management of SMZL, observation as the best approach for patients in group A and rituximab as the best treatment for group B.
摘要：
我们研究的目的是分析对2014年GELTAMOSMZL指南的遵守情况，在脾边缘区淋巴瘤（SMZL）患者中，并根据HPLLs/ABC适应的治疗策略评估结果。2014年至2020年诊断的181名SMZL患者的观察性前瞻性多中心研究。淋巴瘤特异性生存率（LSS），评估了无复合事件生存率(CEFS)和缓解率.纳入分析的168例患者中有57％遵循指南。与脾切除术组相比，利妥昔单抗化疗组和利妥昔单抗组的总反应率更高(p<0.001)。5年总生存率为77%，5年LSS为93%。根据接受的治疗，5年LSS没有差异（p=0.68）。整个系列的5年CEFS为45%，得分A和B之间存在显着差异（p=0.036）。在诊断时或观察后比较接受利妥昔单抗或利妥昔单抗化疗的患者的LSS和无进展生存期时，没有显着差异。我们的数据支持HPLLs/ABC评分作为SMZL管理的实用工具，观察为A组患者的最佳治疗方法，利妥昔单抗为B组患者的最佳治疗方法。
