Dreyling Martin, Ferrero Simone
Department of Medicine III, Hospital of the University LMU München, Germany
Division of Hematology, Department of Molecular Biotechnologies and Health Sciences, University of Torino, Italy.
Haematologica. 2016 Feb;101(2):104-14. doi: 10.3324/haematol.2014.119115.
Based on the profound biological insights of the last years into the molecular pathogenesis of mantle cell lymphoma and the clinical introduction of new targeted drugs, with high efficacy and a good safety profile, the therapeutic scenario for this tumor has been shown to be thoroughly favourable. No longer characterized by a uniformly dismal prognosis, mantle cell lymphoma has been revealed as a spectrum of different diseases, ranging from very indolent cases to highly aggressive and refractory ones. Thus, there is an urgent need to adapt therapy to accommodate the diverse presentations of the disease. High-dose chemotherapy, followed by autologous stem cell transplantation is the current standard of care for younger patients, generally providing high responses and long survival rates, but hampered by acute and long-term toxicity. In addition, some patients may be overtreated, while others could benefit from targeted approaches, based on the new, molecular-directed compounds. Such a personalized treatment based on the specific characteristics of individual patients may be guided by validated prognostic tools, such as the Mantle Cell Lymphoma International Prognostic Index and the Ki-67 Proliferative Index, as well as by early predictors of treatment response, like minimal residual disease analysis. Moreover, mutation screening of distinctive genomic alterations may provide new, predictive biomarkers, with an additional impact on clinical practice. Only after tailoring treatment according to the clinical and biological heterogeneity of the disease the role of transplantation and modern therapeutic options will be redefined in mantle cell lymphoma.
基于近年来对套细胞淋巴瘤分子发病机制的深入生物学认识以及新型靶向药物的临床应用，这些药物疗效高且安全性良好，已表明该肿瘤的治疗前景极为有利。套细胞淋巴瘤不再具有一致的不良预后特征，而是被揭示为一系列不同的疾病，从非常惰性的病例到高度侵袭性和难治性的病例。因此，迫切需要调整治疗方法以适应该疾病的多种表现形式。大剂量化疗后进行自体干细胞移植是年轻患者目前的标准治疗方法，通常能带来高缓解率和长生存率，但受到急性和长期毒性的阻碍。此外，一些患者可能接受了过度治疗，而另一些患者可能会从基于新型分子导向化合物的靶向治疗方法中获益。基于个体患者的特定特征进行这种个性化治疗可能由经过验证的预后工具指导，如套细胞淋巴瘤国际预后指数和Ki-67增殖指数，以及治疗反应的早期预测指标，如微小残留病分析。此外，对独特基因组改变的突变筛查可能提供新的预测生物标志物，对临床实践产生额外影响。只有根据疾病的临床和生物学异质性调整治疗后，移植和现代治疗选择在套细胞淋巴瘤中的作用才会被重新定义。
