University of Heidelberg Hospital, Department of Hematology and Oncology, Germany.
Cancer. 2013 Jan 1;119(1):99-106. doi: 10.1002/cncr.27704. Epub 2012 Jun 26.
In marginal zone lymphoma (MZL), clinical and follow-up data on large cohorts of patients are difficult to obtain. The objective of this single-center, retrospective analysis of a large cohort of 144 patients with MZL was to elucidate the role of prognostic markers, treatments, and outcomes in this disease.
In total, 144 patients were identified who were diagnosed with MZL at the authors' institution between 2003 and 2010. Data on clinical parameters, treatments, response, and survival were analyzed. In addition, the validity of the International Prognostic Index (IPI) and Follicular Lymphoma International Prognostic Index (FLIPI) prognostic scores were tested in patients with MZL.
Among 144 patients with MZL, 96 patients (67%) had extralymph node (extranodal) MZL, 32 patients (22%) had lymph node (nodal) MZL, and 16 patients (11%) had splenic MZL. The 5-year progression-free survival rate was 82% in the nodal MZL group, 88% in the extranodal MZL group, and 74% in the splenic MZL group and did not different between the 3 groups (P = .60). The 5-year overall survival rate was excellent in all 3 MZL groups (nodal MZL, 89%; extranodal MZL, 92%; splenic MZL, 82%; P = .46). In our cohort, the FLIPI score was a significant prognostic marker: The 5-year progression-free survival rate for patients who had FLIPI scores of 0 to 2 (low or intermediate risk) was excellent at 92%, whereas it was only 62% for patients who had FLIPI scores of 3 to 5 (poor risk; P = .003). Similarly, the 5-year overall survival rate for patients who had FLIPI scores of 0 to 2 was 95%, whereas it was only 62% for patients who had FLIPI scores of 3 to 5 (P = .0009).
The FLIPI score had strong prognostic value in patients with MZL. Patients who have low-risk or intermediate-risk FLIPI scores have an excellent prognosis, whereas patients with poor-risk FLIPI scores are candidates for novel treatment approaches.
在边缘区淋巴瘤（MZL）中，获得大量患者的临床和随访数据较为困难。本单中心回顾性分析了 144 例 MZL 患者的大型队列，旨在阐明预后标志物、治疗方法和结局在该疾病中的作用。
共确定了 144 例于 2003 年至 2010 年在作者所在机构诊断为 MZL 的患者。分析了临床参数、治疗方法、反应和生存数据。此外，还测试了国际预后指数（IPI）和滤泡性淋巴瘤国际预后指数（FLIPI）在 MZL 患者中的有效性。
在 144 例 MZL 患者中，96 例（67%）患有结外（结外）MZL，32 例（22%）患有淋巴结（结内）MZL，16 例（11%）患有脾 MZL。结内 MZL 组的 5 年无进展生存率为 82%，结外 MZL 组为 88%，脾 MZL 组为 74%，3 组间差异无统计学意义（P=.60）。3 组 MZL 的 5 年总生存率均非常好（结内 MZL 为 89%；结外 MZL 为 92%；脾 MZL 为 82%；P=.46）。在本队列中，FLIPI 评分是一个显著的预后标志物：FLIPI 评分为 0 至 2 分（低或中危）的患者 5 年无进展生存率为 92%，而评分为 3 至 5 分（高危）的患者仅为 62%（P=.003）。同样，FLIPI 评分为 0 至 2 分的患者 5 年总生存率为 95%，而评分为 3 至 5 分的患者仅为 62%（P=.0009）。
FLIPI 评分在 MZL 患者中具有很强的预后价值。低危或中危 FLIPI 评分的患者预后良好，而高危 FLIPI 评分的患者则是新型治疗方法的候选者。
