Department of Internal Medicine and Gastroenterology, Watanabe Internal Medicine Aoyama Clinic, Niigata 9502002, Japan.
World J Gastroenterol. 2023 Dec 28;29(48):6179-6197. doi: 10.3748/wjg.v29.i48.6179.
Follicular lymphoma (FL) is the most common indolent B-cell lymphoma (BCL) globally. Recently, its incidence has increased in Europe, the United States, and Asia, with the number of gastrointestinal FL cases expected to increase. Genetic abnormalities related to t(14;18) translocation, BCL2 overexpression, NF-κB pathway-related factors, histone acetylases, and histone methyltransferases cause FL and enhance its proliferation. Meanwhile, microRNAs are commonly used in diagnosing FL and predicting patient prognosis. Many clinical trials on novel therapeutics targeting these genetic abnormalities and immunomodulatory mechanisms have been conducted, resulting in a marked improvement in therapeutic outcomes for FL. Although developing these innovative therapeutic agents targeting specific genetic mutations and immune pathways has provided hope for curative options, FL treatment has become more complex, requiring combinatorial therapeutic regimens. However, optimal treatment combinations have not yet been achieved, highlighting the importance of a complete under-standing regarding the pathogenesis of gastrointestinal FL. Accordingly, this article reviews key research on the molecular pathogenesis of nodal FL and novel therapies targeting the causative genetic mutations. Moreover, the results of clinical trials are summarized, with a particular focus on treating nodal and gastrointestinal FLs.
滤泡性淋巴瘤(FL)是全球最常见的惰性 B 细胞淋巴瘤(BCL)。近年来,其在欧洲、美国和亚洲的发病率有所增加,预计胃肠道 FL 病例数量将会增加。与 t(14;18)易位、BCL2 过表达、NF-κB 通路相关因素、组蛋白乙酰转移酶和组蛋白甲基转移酶相关的遗传异常导致了 FL 的发生,并增强了其增殖。同时,microRNAs 常用于诊断 FL 和预测患者预后。许多针对这些遗传异常和免疫调节机制的新型治疗药物的临床试验已经开展,这使得 FL 的治疗效果有了显著改善。尽管开发针对特定遗传突变和免疫途径的这些创新治疗药物为治愈选择提供了希望,但 FL 的治疗变得更加复杂,需要联合治疗方案。然而,尚未达到最佳的治疗组合,这突出表明了全面了解胃肠道 FL 的发病机制的重要性。因此,本文综述了关于结内滤泡性淋巴瘤的分子发病机制和针对致病遗传突变的新型治疗方法的重要研究进展。此外,还总结了临床试验的结果,特别关注了治疗结内和胃肠道 FL 的方法。