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 asTP53mutations, 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）研究进展揭示了该疾病的分子多样性，导致观察到的临床结局存在显著差异。当前预后风险评分体系持续修订，以纳入对MCL机制或生物学理解的新进展。然而，与疾病快速进展和不良生存相关的关键高风险特征，如TP53突变、复杂核型及母细胞样或多形性形态学特征，在现有预后工具中仍未被充分纳入。随着新一代测序等基因组技术的普及，临床医生能够识别作为预后信号和疾病监测参数的特异性基因改变，从而建立能反映MCL异质性的精准风险分层体系。通过对不同MCL生物学行为的深入理解，针对疾病生物学机制的新型疗法——包括布鲁顿酪氨酸激酶抑制剂、BCL-2抑制剂、ROR1抑制剂及双特异性T细胞衔接器——拓宽了复发/难治性MCL的治疗选择。这些干预手段与化学免疫治疗、自体干细胞移植等传统支柱疗法相结合，推动了个体化治疗的实施并改善了生存结局。进一步探索MCL显著的生物学异质性，将有助于深化对这一罕见淋巴瘤亚型的认知、管理与治疗。
Updates on the Biological Heterogeneity of Mantle Cell Lymphoma
肿瘤(癌症)患者之家