PDF(Pubmed)
Abstract：
Mantle cell lymphoma (MCL) is a rare, heterogeneous B-cell non-Hodgkin\'s lymphoma. The standard front-line treatment utilizes chemotherapy, often followed by consolidation with an autologous hematopoietic cell transplant; however, in most patients, the lymphoma will recur and require subsequent treatments. Additionally, mantle cell lymphoma primarily affects older patients and is frequently chemotherapy-resistant, which has further fostered the necessity for new, chemotherapy-free treatment options. In the past decade, targeted therapies in mantle cell lymphoma have been practice-changing as the treatment paradigm shifts further away from relying primarily on cytotoxic agents. Here, we will review the pathophysiology of mantle cell lymphoma and discuss the emergence of targeted, chemotherapy-free treatments aimed at disrupting the abnormal biology driving its lymphomagenesis. Treatments targeting the constitutive activation of NF-kB, Bruton\'s Tyrosine Kinase signaling, and anti-apoptosis will be the primary focus as we discuss their clinical data and toxicities. Our review will also focus primarily on the emergence and use of targeted therapies in the relapsed/refractory setting but will also discuss the emergence of their use in front-line therapy and in combination with other agents.
摘要：
套细胞淋巴瘤（MCL）是一种罕见的，异质性B细胞非霍奇金淋巴瘤。标准的一线治疗利用化疗，通常伴随着自体造血细胞移植的巩固;然而，在大多数患者中，淋巴瘤会复发,需要后续治疗.此外,套细胞淋巴瘤主要影响老年患者，并且经常对化疗耐药，这进一步促进了新的必要性，无化疗的治疗选择。在过去的十年里,套细胞淋巴瘤的靶向治疗已经改变了实践,因为治疗模式已经从主要依赖细胞毒性药物转移到了更远的地方.这里,我们将回顾套细胞淋巴瘤的病理生理学，并讨论靶向，无化疗治疗旨在破坏异常生物学驱动其淋巴生成。靶向NF-kB组成型激活的治疗，布鲁顿酪氨酸激酶信号,抗凋亡将是我们讨论其临床数据和毒性的主要焦点。我们的审查还将主要集中在复发/难治性背景下靶向治疗的出现和使用，但也将讨论其在一线治疗和与其他药物联合使用的出现。
