Department of Medicine III, University Hospital Großhadern/LMU München, Medizinische Klinik und Poliklinik III, Klinikum der Universität, München, Germany.
Division of Hematology, Department of Molecular Biotechnologies and Health Sciences, University of Torino, Torino, Italy.
Leukemia. 2014 Nov;28(11):2117-30. doi: 10.1038/leu.2014.171. Epub 2014 May 23.
Mantle cell lymphoma (MCL) is no longer a hopeless disease. Considered to carry a uniformly dismal prognosis so far, during the last years it has been rediscovered as a heterogeneous clinical and biological entity. Such a complexity has been highlighted by molecular genetics, unraveling different pathways of cell survival and progression. Concurrently, the application of new therapeutic paradigms including rituximab, high-dose cytarabine and stem cell transplantation dramatically improved treatment activity and the introduction of innovative targeted molecules has already led to new patient perspectives. In this completely new and continually evolving landscape, the clinical hemato-oncologist might feel disoriented on what are the best current strategies to handle such a critical disease and the gold standard therapeutic options for MCL. Here we address some burning questions on how to manage MCL patients, spacing from prognostic issues to the dilemma of personalized treatment in different scenarios of the disease: how to diagnose an MCL? Which are the fundamental staging procedures? What are the most reliable prognosticators? Is there a place for watch and wait? Which are the best treatment options for younger, elderly and frail patients? Which patients are addressable to high-dose therapy? What is the role of allogeneic transplantation? What is the most appropriate approach for relapsing disease in different categories of patients? What novelties are going to be introduced in the near future? The practical algorithms here discussed represent an evidence-based approach derived from results of multicenter and randomized trials.
套细胞淋巴瘤(MCL)不再是一种无望的疾病。迄今为止,MCL 被认为预后普遍较差,但近年来,人们重新发现它是一种具有异质性临床和生物学特征的疾病。这种复杂性已被分子遗传学所凸显,揭示了不同的细胞存活和进展途径。与此同时,新的治疗模式的应用,包括利妥昔单抗、高剂量阿糖胞苷和干细胞移植,极大地提高了治疗活性,而创新的靶向药物的引入已经为患者带来了新的前景。在这种全新且不断发展的背景下,临床血液科医生可能会感到困惑,不知道目前处理这种严重疾病的最佳策略是什么,也不知道 MCL 的标准治疗选择是什么。在这里,我们将针对如何管理 MCL 患者提出一些热点问题,从预后问题到不同疾病情况下的个体化治疗困境:如何诊断 MCL?基本的分期程序是什么?最可靠的预后因素是什么?是否有观察等待的空间?对于年轻、老年和体弱患者,最佳的治疗选择是什么?哪些患者适合接受大剂量治疗?异体移植的作用是什么?对于不同类别的患者,复发疾病的最佳处理方法是什么?在不久的将来会有哪些新的进展?这里讨论的实用算法代表了一种基于多中心和随机试验结果的循证方法。