本帖最后由 橙色雨丝 于 2017-1-17 16:35 编辑
来自美国临床肿瘤学会2015年年会的报道：
Background: MZL are uncommon B-cell lymphomas. The outcomes and prognostic factors of MZL remain poorly defined. 背景：边缘区淋巴瘤是比较少见的B细胞淋巴瘤。边缘区淋巴瘤的转归和预后因子尚不明确。
Methods: We retrospectively identified 485 cases ofMZL diagnosed between 1994-2014 from 2 institutions. Diagnoses had been confirmed by expert hematopathologists. We excluded 127 cases because of limited data and overlapping features with other lymphoma subtypes. 方法：我们回顾性的确定了485例在1994年－2014年期间确诊于两家机构的边缘区淋巴瘤。病理经血液学病理专家确认。其中有127例因为数据不全和与其它类型淋巴瘤在特点上有重合而被我们排除。
Results: Of the 358 patients (pts) included in the analysis, 216 (60%) had extranodal MZL (EMZL) with most EMZL arising from stomach (32%), 56 (16%) nodal MZL (NMZL), 64 (18%) splenic MZL (SMZL), and 22(6%) had typical MZL phenotype but were unclassifiable. Median age was 66(range 13-95) years, 171 (48%) pts had stage III/IV disease at diagnosis.B-symptoms were present in 33 (9%) pts and 65 (18%) had > 4 nodal sites (LN)involved. Median time from diagnosis to treatment was 33 (2-1883) days and median duration of follow-up was 40.3 (2.2-236.4) months. 结果：在我们分析的358例病人中，216例（60%）是结外边缘区淋巴瘤，主要是胃MALT（32%），56例（16％）是结内边缘区淋巴瘤，64例（18%）是脾边缘区淋巴瘤，还有22（6%）例是具有边缘区免疫表型但不能分类的淋巴瘤。中位年龄是66岁（13岁－95岁），171例（48%）确诊时具有III／IV期疾病。另外，33例（9%）有B症状，65例（18%）有超过4个淋巴结区侵犯。从确诊到开始治疗的中位时间是33天（2-1883天），中位随访时间是40.3个月（2.2-236.4个月）。
Among treated pts, initial treatment was rituximab (R) (33%), chemotherapy +/- R (22%), radiation (16%),surgery (16%), and antibiotics (13%). Progression-free survival (PFS) and overall survival (OS) for the whole group was 5.15 (95% confidence intervals;C.I. = 2.8-10) and 19 (95% C.I. = 10-19) years respectively and there was nosignificant difference in PFS or OS between EMZL, NMZL, SMZL, or atypical MZL. 在治疗的病人中，初治有33%是美罗华单药，22%是化疗＋／—美罗华，16%是放疗，16%是手术，13%是抗生素。整个组的无进展生存期和总生存期分别为5.15年和19年，几种亚型之间生存率没有明显区别。
On univariate analysis, age >60 (p = 0.002), elevated serum lactate dehydrogenase (LDH) (p = 0.003),involvement of > 4 LN (p = 0.019), follicular lymphoma International Prognostic Index (FLIPI) score (p = 0.0005), and hemoglobin (Hb) level < 12g/dL at presentation (p = 0.003) were associated with inferior OS. In amultivariate analysis, only age (hazard ratio (HR) = 5.48, p = 0.002) and Hb(HR = 2.97, p = 0.009) retained significance. Transformation to aggressive lymphoma occurred in 29 (8%) pts and was associated with high serum LDH andhigher FLIPI at diagnosis. In pts treated with R +/- chemotherapy, Rmaintenance was associated with improved OS (p = 0.04). 通过单变量分析，确诊时年龄>60，LDH升高，超过4个淋巴结区域侵犯，较高滤泡性淋巴瘤国际预后指数，和血红蛋白<12g/dL与不良预后相关。29例（8%）发生了向侵袭性淋巴瘤的转化，与确诊时LDH升高和较高国际预后指数相关。对于经R＋／－化疗治疗的病人，美罗华维持与总生存期改善有关。
Conclusions: In our MZL series, one of the largest reported, prognosis of MZL is good with a median OS of over 19 years. Age >60, Hb < 12 g/dL, elevated LDH, and > 4 LN involved are associated with inferior OS. 结论：我们对边缘区淋巴瘤的研究是已报道的研究中规模最大的之一，结论是边缘区淋巴瘤总体预后良好，中位总生存期超过19年。年龄>60，血红蛋白<12g/dL，LDH升高，超过4个淋巴结区域侵犯与不良预后相关。
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