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）的临床表现和病理特征存在差异，包括高Ki-67表达（>20%）以及大B细胞（LC）数量增多。然而，关于这些变量对MZL患者预后意义的现有数据有限。在这项针对10个中心治疗的MZL患者的多机构回顾性队列研究中，我们评估了Ki-67表达和LC增多与生存期及组织学转化（HT）风险之间的关联。共纳入785例患者（60%为结外MZL，20%为结内MZL，20%为脾MZL）。在440例进行Ki-67染色的患者中，22%存在高Ki-67表达（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患者为7.0年（HR=1.45，95%CI=1.03–2.05）。Ki-67>20%与高LDH水平显著相关。Ki-67增高患者的HT风险高于未增高者（5年风险：9.8% vs 3.87%，p=0.01）。12%的患者活检报告显示存在LC，其中6%的患者LC比例>10%。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。存在LC患者的HT累积风险高于无LC患者（5年风险：9.4% vs 2.9%，p=0.04）。两组患者接受蒽环类药物治疗均未影响PFS或OS。Ki-67染色>20%是生存预后不良的因素，且与LDH升高密切相关。应研究新型疗法在克服MZL高风险特征方面的潜在能力。我们的数据强调了在复发或进展时进行活检的重要性，尤其对于基线Ki-67高和LC增多的患者，因其HT风险增加。
肿瘤(癌症)患者之家