There are three different marginal zone lymphoma (MZLs) entities: the extranodal MZL of mucosa- associated lymphoid tissue, the splenic MZL, and the nodal MZL. The 3 MZLs share common lesions (trisomies of chromosomes 3 and 18, deletions at 6q23), and alterations of the nuclear factor κB pathway are frequent events in all of them, but they also differ in the presence of recurrent translocations, mutations affecting the NOTCH pathway, and the transcription factor Kruppel-like factor 2 (KLF2) or the receptor-type protein tyrosine phosphatase delta (PTPRD). This review outlines the most recent and main advances in our understanding of the genetics and biology of MZLs and summarizes the clinical activity of the novel therapeutic approaches targeting the main druggable pathways. The current principles of the standard management of MZL at different anatomic sites are also discussed.

存在三种不同的边缘区淋巴瘤（MZL）实体：粘膜相关淋巴组织的结外MZL，脾MZL和淋巴结MZL。3个MZL具有共同的病变（3号染色体和18号染色体的三体性，在6q23处缺失），并且核因子κB通路的改变都是它们中的常见事件，但是它们在复发性易位，影响NOTCH的突变方面也存在差异途径，转录因子Kruppel样因子2（KLF2）或受体型蛋白酪氨酸磷酸酶δ（PTPRD）。这篇综述概述了我们对MZLs的遗传学和生物学的理解的最新和主要进展，并总结了针对主要药物途径的新型治疗方法的临床活性。还讨论了在不同解剖部位的MZL标准管理的当前原则。