Rosen Steven T, Link Brian K, Fowler Nathan H
Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Clin Adv Hematol Oncol. 2013 Nov;11(11 Suppl 18):1-19.
Mantle cell lymphoma is one of the most challenging hematologic malignancies, owing to an aggressive disease course, a high rate of relapse, and lack of standard of care. In the United States, mantle cell lymphoma accounts for approximately 6% of all newly diagnosed cases of non-Hodgkin lymphoma. Because most patients are initially diagnosed with advanced-stage disease, they are often symptomatic at presentation. Common features include widespread lymphadenopathy and splenomegaly, as well as bone marrow infiltration. Leukemic involvement is found in 20% to 30% of patients. The disease course can be highly variable. Some patients may have very aggressive disease, whereas others may have a much more indolent course. The optimal frontline therapy remains undefined. Strategies include chemotherapy, immunotherapy, radioimmunotherapy, stem cell transplantation, and novel biologic agents. Although mantle cell lymphoma often responds well to frontline chemotherapy, the responses are not durable and often of relatively short duration. Effective treatment options in the frontline setting have included the addition of rituximab to bendamustine. Once mantle cell lymphoma has entered the relapsed/refractory stage, it becomes more difficult to treat. Bortezomib and lenalidomide are approved for treatment of relapsed/refractory mantle cell lymphoma. The novel Bruton’s tyrosine kinase inhibitor ibrutinib appears to be highly active in relapsed/refractory mantle cell lymphoma. Other agents in clinical trials include cladribine, idelalisib, and IPI-145.
套细胞淋巴瘤是最具挑战性的血液系统恶性肿瘤之一，因其病程侵袭性强、复发率高且缺乏标准治疗方案。在美国，套细胞淋巴瘤约占所有新诊断非霍奇金淋巴瘤病例的6%。由于大多数患者最初被诊断为晚期疾病，他们在就诊时通常有症状。常见特征包括广泛的淋巴结病和脾肿大，以及骨髓浸润。20%至30%的患者有白血病累及。疾病进程差异很大。一些患者可能患有侵袭性很强的疾病，而另一些患者的病程可能较为惰性。最佳一线治疗方案仍未明确。治疗策略包括化疗、免疫治疗、放射免疫治疗、干细胞移植和新型生物制剂。虽然套细胞淋巴瘤通常对一线化疗反应良好，但反应并不持久，且持续时间往往较短。一线治疗的有效选择包括在苯达莫司汀基础上加用利妥昔单抗。一旦套细胞淋巴瘤进入复发/难治阶段，治疗就变得更加困难。硼替佐米和来那度胺被批准用于治疗复发/难治性套细胞淋巴瘤。新型布鲁顿酪氨酸激酶抑制剂依鲁替尼在复发/难治性套细胞淋巴瘤中似乎具有很高的活性。临床试验中的其他药物包括克拉屈滨、艾代拉里斯和IPI-145。
