University of Heidelberg Hospital, Heidelberg, Germany.
Ann Hematol. 2012 Nov;91(11):1765-72. doi: 10.1007/s00277-012-1534-y. Epub 2012 Aug 29.
In most patients, mantle cell lymphoma (MCL) shows an aggressive clinical course with a continuous relapse pattern and a median survival of only 3-5 years. In the current study generation of the European MCL Network, the addition of high-dose Ara-C to R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone)-like regimen followed by myeloablative consolidation achieved a significant improvement of progression-free survival in younger patients. In elderly patients, rituximab maintenance led to a marked prolongation of remission duration. Emerging strategies include mammalian target of rapamycin (mTOR) inhibitors, proteasome inhibitors, immune modulatory drugs, Bruton's tyrosine kinase inhibitors and others, all based on the dysregulated control of cell cycle machinery and impairment of several apoptotic pathways. Combination strategies are currently being investigated in numerous trials, but their introduction into clinical practice and current treatment algorithms remains a challenge. In the current survey, the application of the molecular targeted compounds were collected and evaluated by a representative national network of 14 haematological institutions. Optimised strategies are recommended for clinical routine. Future studies will apply individualised approaches according to the molecular risk profile of the patient.
在大多数患者中，套细胞淋巴瘤（MCL）表现出侵袭性临床病程，具有持续复发模式，中位生存期仅为 3-5 年。在欧洲 MCL 网络的当前研究中，在类似于 R-CHOP（利妥昔单抗、环磷酰胺、多柔比星、长春新碱和泼尼松）的方案中添加高剂量阿糖胞苷，随后进行清髓性巩固，可显著改善年轻患者的无进展生存期。在老年患者中，利妥昔单抗维持治疗导致缓解持续时间显著延长。新兴策略包括哺乳动物雷帕霉素靶蛋白（mTOR）抑制剂、蛋白酶体抑制剂、免疫调节剂、布鲁顿酪氨酸激酶抑制剂等，所有这些策略都基于细胞周期机制的失调控制和几个凋亡途径的损害。目前正在对许多试验中的联合策略进行研究，但将其引入临床实践和当前的治疗算法仍然是一个挑战。在当前的调查中，通过代表全国 14 个血液学机构的网络收集并评估了分子靶向化合物的应用。为临床常规推荐了优化策略。未来的研究将根据患者的分子风险特征采用个体化方法。
