Ip Andrew, Kabat Maciej, Fogel Lindsay, Alkhatatneh Hassan, Voss Jason, Gupta Amolika, Della Pia Alexandra, Leslie Lori A, Feldman Tatyana, Albitar Maher, Goy Andre H
Department of Oncology, Hackensack Meridian School of Medicine, Nutley, NJ 07110, USA.
John Theurer Cancer Center, Hackensack Meridian Health, Hackensack, NJ 07601, USA.
Cancers (Basel). 2025 Feb 19;17(4):696. doi: 10.3390/cancers17040696.
Advancements in mantle cell lymphoma (MCL) have illuminated the disease's molecular diversity, leading to a wide variation in the outcomes observed in MCL. Current prognostic risk scores are continuously revised to incorporate new updates in the mechanistic or biologic understanding of MCL. Nevertheless, key high-risk features of MCL associated with rapid disease progression and poor survival, such as mutations, complex karyotypes, and blastoid or pleomorphic morphologies, remain absent from available prognostic tools. The greater accessibility of genomic technologies, such as next-generation sequencing (NGS), has enabled clinicians to identify specific genetic alterations that serve as prognostic signals and disease monitoring parameters, cultivating accurate risk profiling that is illustrative of MCL heterogeneity. Through an increased understanding of distinct MCL behaviors, novel therapies that mechanistically target disease biology, including Bruton's tyrosine kinase inhibitors, BCL-2 inhibitors, ROR1 inhibitors, and bispecific T-cell engagers, have broadened the treatment armamentarium for relapsed/refractory MCL cases. These interventions, in addition to chemoimmunotherapy and autologous stem cell transplantation mainstays, confer the individualization of treatment and improved survival outcomes. Further exploration of the considerable biological heterogeneity of MCL can enhance knowledge, management, and the treatment of this rare lymphoma subtype.
套细胞淋巴瘤(MCL)的进展揭示了该疾病的分子多样性,导致MCL患者的预后差异很大。当前的预后风险评分不断修订,以纳入对MCL机制或生物学理解的新进展。然而,现有预后工具仍未涵盖与疾病快速进展和生存不良相关的MCL关键高危特征,如突变、复杂核型以及母细胞样或多形性形态。基因组技术(如下一代测序(NGS))的更广泛应用,使临床医生能够识别作为预后信号和疾病监测参数的特定基因改变,从而建立准确的风险谱,以体现MCL的异质性。通过对不同MCL行为的深入了解,以疾病生物学为机制靶点的新型疗法,包括布鲁顿酪氨酸激酶抑制剂、BCL-2抑制剂、ROR1抑制剂和双特异性T细胞衔接器,拓宽了复发/难治性MCL病例的治疗手段。这些干预措施,除了化疗免疫疗法和自体干细胞移植等主要治疗方法外,还实现了治疗的个体化并改善了生存结果。对MCL显著生物学异质性的进一步探索,可以增进对这种罕见淋巴瘤亚型的认识、管理和治疗。