University of Virginia School of Medicine, Charlottesville, VA, USA.
Clin Lymphoma Myeloma Leuk. 2010 Oct;10(5):336-46. doi: 10.3816/CLML.2010.n.066.
Mantle cell lymphoma (MCL) is regarded as an aggressive lymphoid malignancy that exhibits varied clinical behavior and prognoses, reflecting the biologic heterogeneity of the disease. In most cases, patients with MCL achieve a shorter median survival compared with more common B-cell lymphomas, such as follicular lymphoma, and are less likely to achieve a durable response with chemotherapy. Currently, there is no defined standard of care for patients with MCL. Rituximab-containing immunochemotherapy strategies are commonly used, but the addition of rituximab to conventional induction chemotherapy has produced suboptimal responses that are relatively short-lived and have not resulted in a survival advantage. Further intensification of the chemotherapy component, including autologous stem cell transplantation, has increased response and survival rates but has not proven to be curative while being associated with higher toxicity. Clearly, there is a need for developing novel agents and strategies that will improve clinical outcomes for patients with MCL. Targeted therapies and new cytotoxic agents are showing great promise and may have a role in maintenance and/or initial therapy. This summary highlights current challenges in the management of MCL, and outlines expert perspectives, key questions, and future directions. For the third consecutive year, a panel of global experts in MCL assembled to deliberate on topical issues in MCL including advances in pathobiology, strategies for risk-adapted therapy, front-line treatment options, consolidation approaches, and novel therapeutic strategies. The proceedings of this workshop, held December 3, 2009 in New Orleans, LA, are summarized here. It must be emphasized that this synopsis is not meant to serve as an exhaustive review of MCL biology and management, but is a distillation of the expert discussions, highlighting key questions and future directions identified.
套细胞淋巴瘤(MCL)被认为是一种侵袭性的淋巴恶性肿瘤,表现出不同的临床行为和预后,反映了该病的生物学异质性。在大多数情况下,与更常见的 B 细胞淋巴瘤(如滤泡性淋巴瘤)相比,MCL 患者的中位生存期较短,并且不太可能通过化疗获得持久缓解。目前,MCL 患者尚无明确的标准治疗方法。含利妥昔单抗的免疫化疗方案通常被采用,但利妥昔单抗联合常规诱导化疗产生的反应不理想,且持续时间较短,并未带来生存优势。进一步强化化疗部分,包括自体造血干细胞移植,虽然提高了反应率和生存率,但并未证明具有治愈作用,而且与更高的毒性相关。显然,需要开发新的药物和策略,以改善 MCL 患者的临床结果。靶向治疗和新型细胞毒药物显示出巨大的潜力,可能在维持和/或初始治疗中发挥作用。本综述强调了 MCL 管理中的当前挑战,并概述了专家观点、关键问题和未来方向。连续第三年,一组 MCL 领域的全球专家聚集在一起,讨论 MCL 中的热门话题,包括病理生物学进展、风险适应治疗策略、一线治疗选择、巩固方法和新的治疗策略。该研讨会于 2009 年 12 月 3 日在路易斯安那州新奥尔良举行,现将会议记录摘要如下。必须强调的是,本概要并不是要全面回顾 MCL 的生物学和管理,而是对专家讨论的提炼,突出了确定的关键问题和未来方向。