Service d'hématologie, Université Paris Descartes, Assistance des hôpitaux de Paris, Hôpital Necker, Paris, France.
Semin Hematol. 2011 Jul;48(3):194-207. doi: 10.1053/j.seminhematol.2011.06.002.
Mantle cell lymphoma (MCL) is an individualized entity that is well characterized at the molecular level and considered to be a disease of elderly patients. However, about half of patients are less than 65 years of age and may benefit from intensive therapies. Although MCL has been considered during the last three decades as an incurable disease with current chemotherapy regimens, in young patients recent intense chemo-immunotherapy (CIT) induction regimens including high-dose cytarabine with consolidation with autologous stem cell transplantation (ASCT) have increased significantly the outcome of patients with the disease; some may experience long-term survival free of disease and may even be cured. In addition, new drugs targeting some pathways, including molecular alterations of the disease, are being progressively incorporated into the therapeutic armamentarium of the disease and will certainly contribute to further improve prognosis. In the near future, more individualized approaches are foreseen that will take into account risk factors present at diagnosis, biomarkers representative of the molecular alterations, as well as quality of the response assessed by molecular residual diseases analysis. In this review, we discuss the current therapeutic approaches with classical CITs, the role of autologous and allogeneic stem cell transplantation, and the main new drugs that target major molecular pathways alterations of the disease, as well as their positioning during induction, consolidation, and maintenance in first-line treatment and in relapsing younger patients with MCL.
套细胞淋巴瘤（Mantle cell lymphoma，MCL）是一种具有明确分子特征的个体化实体肿瘤，被认为是老年患者的疾病。然而，约一半的患者年龄小于 65 岁，可能受益于强化治疗。尽管在过去三十年中，由于目前的化疗方案，MCL 被认为是一种无法治愈的疾病，但在年轻患者中，最近包括大剂量阿糖胞苷联合自体干细胞移植（Autologous stem cell transplantation，ASCT）巩固治疗在内的强化化疗免疫治疗（Chemo-immunotherapy，CIT）诱导方案显著提高了患者的预后；一些患者可能长期无病生存，甚至可能被治愈。此外，针对某些通路的新药，包括疾病的分子改变，正在逐渐被纳入疾病的治疗武器库，肯定会进一步改善预后。在不久的将来，预计会采用更多个体化的方法，这些方法将考虑到诊断时存在的风险因素、代表分子改变的生物标志物，以及通过分子残留疾病分析评估的反应质量。在这篇综述中，我们讨论了目前使用经典 CIT 的治疗方法、自体和异体干细胞移植的作用，以及针对疾病主要分子通路改变的主要新药，以及它们在一线治疗中诱导、巩固和维持阶段的定位，以及在复发的年轻 MCL 患者中的定位。
