Di Rocco Alice, Petrucci Luigi, Assanto Giovanni Manfredi, Martelli Maurizio, Pulsoni Alessandro
Hematology, Departement of Traslational and Precision Medicine, Sapienza University of Rome, 00161 Rome, Italy.
Cancers (Basel). 2022 Mar 29;14(7):1742. doi: 10.3390/cancers14071742.
Extranodal Marginal Zone Lymphoma (EMZL lymphoma) is an indolent B-cell lymphoma with a median age at diagnosis of about 60 years. It accounts for 7-8% of all B-cell lymphomas. It can occur in various extranodal sites, including stomach, lung, ocular adnexa, and skin; furthermore, the disseminated disease can be found in 25-50% of cases. Several infectious agents, such as () in the case of gastric Mucosa Associated Lymphoid Tissue (MALT) Lymphoma, can drive the pathogenesis of this cancer, through the autoantigenic stimulation of T cells, but there may also be other factors participating such autoimmune diseases. Initial staging should include total body computed tomography, bone marrow aspirate, and endoscopic investigation if indicated. Fluorescence in situ hybridization (FISH), should be performed to detect the presence of specific chromosomal translocations involving the and genes, which leads to the activation of the signaling pathway. Depending on the location and dissemination of the disease, different therapeutic choices may include targeted therapy against the etiopathogenetic agent, radiotherapy, immunochemotherapy, and biological drugs. The purpose of this review is to illustrate the complex biology and the diagnosis of this disease and to better define new treatment strategies.
结外边缘区淋巴瘤（EMZL淋巴瘤）是一种惰性B细胞淋巴瘤，诊断时的中位年龄约为60岁。它占所有B细胞淋巴瘤的7-8%。它可发生于各种结外部位，包括胃、肺、眼附属器和皮肤；此外，25-50%的病例可出现播散性疾病。几种感染因子，如胃黏膜相关淋巴组织（MALT）淋巴瘤中的（此处原文括号内容缺失），可通过T细胞的自身抗原刺激驱动这种癌症的发病机制，但也可能有其他因素参与，如自身免疫性疾病。初始分期应包括全身计算机断层扫描、骨髓穿刺检查，如有必要还应进行内镜检查。应进行荧光原位杂交（FISH）以检测涉及 和 基因的特定染色体易位的存在，这会导致 信号通路的激活。根据疾病的部位和播散情况，不同的治疗选择可能包括针对病因发病因素的靶向治疗、放疗、免疫化疗和生物药物。本综述的目的是阐述这种疾病的复杂生物学特性和诊断方法，并更好地确定新的治疗策略。
