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套细胞淋巴瘤:不断发展的治疗策略。

Mantle cell lymphoma: evolving management strategies.

机构信息

Hematopathology Section, Department of Pathology, Hospital Clinic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Barcelona, Spain; and.

Plymouth University Peninsula Schools of Medicine and Dentistry and Derriford Hospital, Plymouth, United Kingdom.

出版信息

Blood. 2015 Jan 1;125(1):48-55. doi: 10.1182/blood-2014-05-521898. Epub 2014 Dec 11.

Abstract

Mantle cell lymphoma (MCL) is a rare and aggressive form of non-Hodgkin's lymphoma that generally affects older individuals and continues to have one of the worst outcomes of all the lymphomas. Over the last decade, there has been a widespread adoption of cytarabine-based therapy in younger patients, and the incorporation of rituximab into chemotherapeutic regimens has become an evidence-based standard of care. However MCL remains a largely incurable disease, and following relapse, it can be a challenge to manage. Although it is possible to define prognosis reliably, there are, as yet, no clear diagnostic or response-adjusted parameters that can help to guide therapeutic decisions. However, there are a number of highly active targeted therapies that are moving into the clinic that are set to transform the therapeutic paradigm for this disease in the very near future. This review will explore the molecular pathogenesis of MCL and the current and evolving therapeutic strategies for this disease.

摘要

套细胞淋巴瘤(Mantle cell lymphoma,MCL)是一种罕见且侵袭性较强的非霍奇金淋巴瘤,通常影响老年人,并且仍然是所有淋巴瘤中预后最差的一种。在过去十年中,基于阿糖胞苷的治疗方案已在年轻患者中广泛应用,利妥昔单抗与化疗方案联合应用已成为循证治疗标准。然而,MCL 仍然是一种基本无法治愈的疾病,在复发后,其治疗极具挑战。虽然可以可靠地确定预后,但目前尚无明确的诊断或反应调整参数可帮助指导治疗决策。然而,有许多高效的靶向治疗药物正在进入临床应用,有望在不久的将来改变该疾病的治疗模式。本综述将探讨 MCL 的分子发病机制以及该疾病目前和不断发展的治疗策略。

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