Marginal zone lymphoma (MZL) can have varied presentations and pathologic features, including high Ki-67 expression ( > 20%) as well as increased numbers of large B cells (LC). However, there are limited data available demonstrating the prognostic significance of these variables in patients with MZL. In this multi-institutional retrospective cohort study of patients with MZL treated at 10 centers, we evaluated the association between the presence of Ki-67 expression and increased LCs on survival and risk of histologic transformation (HT). A total of 785 patients were included (60% with extranodal MZL, 20% with nodal MZL, and 20% with splenic MZL). Among the 440 patients with Ki-67 staining, 22% had high Ki-67 (Ki-67 >20%). The median progression-free survival (PFS) for patients with high Ki-67 was 5.4 years compared to 7.0 years for patients with low Ki-67 (HR = 1.45, 95%CI = 1.03–2.05). Ki-67 > 20% strongly correlated with high LDH level. The risk of HT was higher in patients with increased Ki-67 than those without (5-year risk, 9.8% vs 3.87%, p = 0.01). Twelve percent of patients had LC reported on biopsy with 6% having >10% LC. The presence of LC was associated with high Ki-67 (p < 0.001), but not associated with shorter PFS or overall survival (OS). The cumulative risk for HT was higher in patients with LC compared to those without LC (5-year risk, 9.4% vs 2.9%, p = 0.04). Receipt of anthracycline-based therapy did not impact PFS or OS in either group. Ki-67 staining >20% was a prognostic factor for worse survival and strongly correlated with elevated LDH. Novel therapies should be investigated for their potential ability to overcome the high-risk features in MZL. Our data reinforce the importance of obtaining biopsies at relapse or progression, particularly in patients with baseline high Ki-67 and increased LCs, given their increased risk for HT.

边缘区淋巴瘤 （MZL） 可有多种表现和病理特征，包括 Ki-67 高表达 （> 20%） 以及大 B 细胞 （LC） 数量增加。然而，可用的数据有限，证明这些变量对 MZL 患者的预后意义。在这项对 10 个中心接受治疗的 MZL 患者的多机构回顾性队列研究中，我们评估了 Ki-67 表达的存在与 LC 增加对生存率和组织学转化 （HT） 风险的相关性。共纳入 785 例患者 （60% 结外 MZL，20% 结节 MZL，20% 脾 MZL）。440 例 Ki-67 染色患者中，22% 为 Ki-67 （Ki-67 >20%）。高 Ki-67 患者的中位无进展生存期 （PFS） 为 5.4 年，而低 Ki-67 患者的中位无进展生存期 （PFS） 为 7.0 年 （HR = 1.45,95% CI = 1.03-2.05）。Ki-67 > 20% 与高 LDH 水平强相关。Ki-67 升高的患者发生 HT 的风险高于无 Ki-67 升高的患者 （5 年风险，9.8% vs 3.87%，p = 0.01）。12% 的患者在活检中报告了 LC，其中 6% 的患者报告了 >10% LC。LC 的存在与高 Ki-67 相关 （p < 0.001），但与较短的 PFS 或总生存期 （OS） 无关。与无 LC 患者相比，LC 患者发生 HT 的累积风险更高 （5 年风险，9.4% vs 2.9%，p = 0.04）。接受基于蒽环类药物的治疗对两组的 PFS 或 OS 均无影响。Ki-67 染色 >20% 是生存率较差的预后因素，与 LDH 升高密切相关。应研究新疗法克服 MZL 中高风险特征的潜在能力。 我们的数据强调了在复发或进展时进行活检的重要性，特别是对于基线高 Ki-67 和 LCs 增加的患者，因为他们的 HT 风险增加。