Denlinger Nathan M, Epperla Narendranath, William Basem M
Department of Internal Medicine, The Ohio State University Comprehensive Cancer Center (OSUCCC-James), The Ohio State University, Columbus, OH, USA.
Cancer Manag Res. 2018 Mar 27;10:615-624. doi: 10.2147/CMAR.S133291. eCollection 2018.
Marginal zone lymphomas (MZLs) consist of a diverse family of malignancies, which are derived from B-cells. The disease subtypes are recognized extranodal, nodal, and splenic MZLs. The disease characteristics, clinical course, and treatment vary considerably based on the site of involvement. In 2017, the US Food and Drug Administration approved ibrutinib, a first in class Bruton's tyrosine kinase inhibitor that revolutionized the care of chronic lymphocytic leukemia patients; for, the treatment of relapsed/refractory MZL based on pivotal open-label Phase II trial demonstrated an overall response rate of 48%, with a complete response rate of 3%, median progression-free survival of 14.2 months, and median overall survival not yet reached at a median follow-up of 19.4 months. In this review, we aim to summarize the current conundrums in the management of MZL and the evolving role of ibrutinib in the treatment of MZL.
边缘区淋巴瘤(MZL)由多种源自B细胞的恶性肿瘤组成。该疾病亚型包括结外、结内和脾MZL。根据受累部位的不同,疾病特征、临床病程和治疗方法差异很大。2017年,美国食品药品监督管理局批准了伊布替尼,这是首个获批的布鲁顿酪氨酸激酶抑制剂,它彻底改变了慢性淋巴细胞白血病患者的治疗方式;基于关键的开放标签II期试验,伊布替尼用于治疗复发/难治性MZL的总体缓解率为48%,完全缓解率为3%,中位无进展生存期为14.2个月,在中位随访19.4个月时中位总生存期尚未达到。在本综述中,我们旨在总结MZL管理中的当前难题以及伊布替尼在MZL治疗中不断演变的作用。