Department of Oncology/Hematology, School of Medicine, Loma Linda University, Loma Linda, CA 92354, USA.
Department of Internal Medicine, School of Medicine, University of California San Francisco-Fresno, Fresno, CA 93701, USA.
Int J Mol Sci. 2023 Jun 16;24(12):10207. doi: 10.3390/ijms241210207.
Mantle cell lymphoma (MCL) is a type of non-Hodgkin lymphoma (NHL) characterized by a hallmark translocation of t (11; 14). CD10 negativity has been used to differentiate MCL from other NHL types; however, recently, there has been an increase in the number of reported cases of CD10-positive MCL. This warrants further investigation into this rarer immunophenotype and its clinical significance. BCL6, which is a master transcription factor for the regulation of cell proliferation and key oncogene in B cell lymphomagenesis, has been reported to have co-expression with CD10 in MCL. The clinical significance of this aberrant antigen expression remains unknown. We conducted a systematic review by searching four databases and selected five retrospective analyses and five case series. Two survival analyses were conducted to determine if BCL6 positivity conferred a survival difference: 1. BCL6+ vs. BCL6- MCL. 2. BCL6+/CD10+ vs. BCL6-/CD10+ MCL. Correlation analysis was conducted to determine if BCL6 positivity correlated with the Ki67 proliferation index (PI). Overall survival (OS) rates were performed by the Kaplan-Meier method and log-rank test. Our analyses revealed that BCL6+ MCL had significantly shorter overall survival (median OS: 14 months vs. 43 months; = 0.01), BCL6+/CD10+ MCL had an inferior outcome vs. BCL6+/CD10- MCL (median OS: 20 months vs. 55 months = 0.1828), BCL6+ MCL had significantly higher percentages of Ki67% (Ki67% difference: 24.29; = 0.0094), and BCL6 positivity had a positive correlation with CD10+ status with an odds ratio 5.11 (2.49, 10.46; = 0.0000286). Our analysis showed that BCL6 expression is correlated with CD10 positivity in MCL, and BCL6 expression demonstrated an inferior overall survival. The higher Ki67 PI in BCL6+ MCL compared to BCL6- MCL further supports the idea that the BCL6+ immunophenotype may have prognostic value in MCL. MCL management should consider incorporating prognostic scoring systems adjusted for BCL6 expression. Targeted therapies against BCL6 may offer potential therapeutic options for managing MCL with aberrant immunophenotypes.
套细胞淋巴瘤（MCL）是一种非霍奇金淋巴瘤（NHL），其特征是存在标志性易位 t（11；14）。CD10 阴性已被用于将 MCL 与其他 NHL 类型区分开来；然而，最近报告的 CD10 阳性 MCL 病例数量有所增加。这需要进一步研究这种罕见的免疫表型及其临床意义。BCL6 是细胞增殖调控的主要转录因子，也是 B 细胞淋巴瘤发生的关键癌基因，据报道在 MCL 中与 CD10 共表达。这种异常抗原表达的临床意义尚不清楚。我们通过搜索四个数据库进行了系统评价，并选择了五篇回顾性分析和五篇病例系列。进行了两项生存分析以确定 BCL6 阳性是否会导致生存差异：1. BCL6+ 与 BCL6- MCL。2. BCL6+/CD10+ 与 BCL6-/CD10+ MCL。进行相关性分析以确定 BCL6 阳性是否与 Ki67 增殖指数（PI）相关。通过 Kaplan-Meier 方法和对数秩检验进行总体生存率（OS）分析。我们的分析表明，BCL6+ MCL 的总生存率明显更短（中位 OS：14 个月 vs. 43 个月； = 0.01），BCL6+/CD10+ MCL 的预后明显差于 BCL6+/CD10- MCL（中位 OS：20 个月 vs. 55 个月； = 0.1828），BCL6+ MCL 的 Ki67% 百分比明显更高（Ki67%差异：24.29； = 0.0094），BCL6 阳性与 CD10+ 状态呈正相关，优势比为 5.11（2.49，10.46； = 0.0000286）。我们的分析表明，BCL6 在 MCL 中与 CD10 阳性相关，BCL6 表达显示出较差的总体生存率。与 BCL6- MCL 相比，BCL6+ MCL 中的 Ki67 PI 更高，这进一步支持了 BCL6+ 免疫表型在 MCL 中可能具有预后价值的观点。MCL 管理应考虑纳入针对 BCL6 表达调整的预后评分系统。针对 BCL6 的靶向治疗可能为管理具有异常免疫表型的 MCL 提供潜在的治疗选择。
