Division of Hematology, Oncology Institute of Southern Switzerland, Bellinzona, Switzerland.
Laboratory of Experimental Hematology, Institute of Oncology Research, Bellinzona, Switzerland.
Curr Oncol Rep. 2021 Feb 12;23(3):26. doi: 10.1007/s11912-020-01001-x.
Richter syndrome (RS) is an uncommon but aggressive evolution of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). RS is an unmet clinical need in the field of CLL. Recent advances in understanding the biology of this condition provide the rationale for testing new therapeutic concepts in order to improve the outcome of patients developing RS, which is so far poor. In this review, we summarize disease characteristics and available therapeutic options for RS.
Current regimens with novel agents in monotherapy have shown little impact on survival. Nevertheless, the better reported outcome for RS has been achieved with the combination of chemo-immunotherapy with a novel agent, confirming the synergistic effect of the approaches. Still, the frailty of this population may impose a less toxic management leaving most patients with no reasonable therapeutic option. Treatment options for RS need to be further expanded. Preclinical models in current development may allow to explore actionable pathways and identify new drug targeted combinations.
Richter 综合征（RS）是慢性淋巴细胞白血病/小淋巴细胞淋巴瘤（CLL/SLL）的一种罕见但侵袭性的演变。RS 是 CLL 领域未满足的临床需求。对该疾病生物学的最新认识为测试新的治疗概念提供了依据，以改善发生 RS 的患者的预后，目前 RS 患者的预后仍然较差。在这篇综述中，我们总结了 RS 的疾病特征和可用的治疗选择。
目前新型单药治疗方案对生存的影响不大。然而，新型药物联合化疗免疫治疗的报告结果显示 RS 预后更好，证实了这些方法的协同作用。尽管如此，该人群的脆弱性可能需要采用毒性较小的治疗方法，导致大多数患者没有合理的治疗选择。需要进一步扩大 RS 的治疗选择。目前正在开发的临床前模型可能允许探索可行的途径并确定新的靶向药物联合治疗。
