Division of Hospital Medicine, Miller School of Medicine, University of Miami, Miami, Florida.
Divison of Hematology, Sylvester Comprehensive Cancer Center, Miller School of Medicine, University of Miami, Miami, Florida.
Cancer. 2020 Nov 1;126(21):4706-4716. doi: 10.1002/cncr.33117. Epub 2020 Aug 7.
Splenic marginal zone lymphoma (SMZL) is a rare tumor without a uniform treatment approach. The authors describe a large population-based study evaluating survival outcomes of patients with SMZL according to the treatment received.
From the Surveillance, Epidemiology, and End Results database, patients were selected who had SMZL diagnosed from 1999 to 2016. Observation, splenectomy, chemotherapy, and splenectomy with chemotherapy were the evaluated treatment strategies. Cox and Fine and Gray regression models were used to evaluate overall and SMZL-specific survival, respectively.
In total, 1671 patients were selected for the analysis. Most patients were aged >60 years (71.3%), White (89.7%), and non-Hispanic (91.7%). Transformation to diffuse large B-cell lymphoma (DLBCL) occurred in 71 patients (4.2%), and the 10-year transformation rate was 8.6% (95% CI, 6.6%-10.9%). In multivariable analysis, shorter SMZL-specific survival was associated with age ≥60 years (subdistribution hazard ratio [SHR], 1.85; 95% CI, 1.40-2.45; P < .001), Hispanic ethnicity (SHR, 1.50; 95% CI, 1.06-2.13; P = .023), DLBCL transformation (SHR, 2.10; 95% CI, 1.48-2.97; P < .001), and the presence of B-symptoms (SHR, 1.67; 95% CI, 1.23-2.27; P < .001). Compared with splenectomy, observation (SHR, 0.92; 95% CI, 0.67-1.28; P = .636), chemotherapy only (SHR, 1.28; 95% CI, 0.93-1.76; P = .127), and splenectomy plus chemotherapy (SHR, 1.43; 95% CI, 0.96-2.13; P = .089) showed no significant differences in SMZL-specific survival. Predictors of shorter overall survival were age ≥60 years (hazard ratio, 2.98; 95% CI, 2.37-3.76; P < .001) and the presence of B-symptoms (hazard ratio, 1.33; 95% CI, 1.06-1.67; P = .014).
There were no significant differences in overall or SMZL-specific survival by treatment strategy. Older age, Hispanic ethnicity, DLBCL transformation, and B-symptoms were associated with a worse prognosis.
脾边缘区淋巴瘤（SMZL）是一种罕见的肿瘤，没有统一的治疗方法。作者描述了一项基于人群的大型研究，根据所接受的治疗评估了 SMZL 患者的生存结果。
从监测、流行病学和最终结果数据库中，选择了 1999 年至 2016 年间诊断为 SMZL 的患者。观察、脾切除术、化疗以及脾切除术联合化疗是评估的治疗策略。Cox 和 Fine 和 Gray 回归模型分别用于评估总体和 SMZL 特异性生存。
共选择了 1671 例患者进行分析。大多数患者年龄大于 60 岁（71.3%），白人（89.7%）和非西班牙裔（91.7%）。71 例（4.2%）发生弥漫性大 B 细胞淋巴瘤（DLBCL）转化，10 年转化率为 8.6%（95%CI，6.6%-10.9%）。多变量分析显示，SMZL 特异性生存较短与年龄≥60 岁（亚分布风险比[SHR]，1.85；95%CI，1.40-2.45；P<.001）、西班牙裔（SHR，1.50；95%CI，1.06-2.13；P=.023）、DLBCL 转化（SHR，2.10；95%CI，1.48-2.97；P<.001）和 B 症状存在（SHR，1.67；95%CI，1.23-2.27；P<.001）相关。与脾切除术相比，观察（SHR，0.92；95%CI，0.67-1.28；P=.636）、仅化疗（SHR，1.28；95%CI，0.93-1.76；P=.127）和脾切除术联合化疗（SHR，1.43；95%CI，0.96-2.13；P=.089）在 SMZL 特异性生存方面无显著差异。总生存较短的预测因素为年龄≥60 岁（风险比，2.98；95%CI，2.37-3.76；P<.001）和 B 症状存在（风险比，1.33；95%CI，1.06-1.67；P=.014）。
治疗策略对总体或 SMZL 特异性生存无显著影响。年龄较大、西班牙裔、DLBCL 转化和 B 症状与预后较差相关。
