Richter transformation (RT) is a rare albeit devastating complication of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL). RT is defined as an aggressive lymphoma, typically diffuse large B-cell lymphoma, in the setting of CLL. A clonal relationship to the preceding CLL clone is detected in the majority of RT cases and confers more aggressive clinicopathologic kinetics, resistance to standard chemoimmunotherapy regimens, and inferior survival. Taken together, these considerations precipitate a significant unmet need for novel therapeutic strategies that improve the outcomes of patients with RT. Through this review, we will explore current data on emerging regimens targeting BTK, BCL-2, CD79, CD20, PI3K, and PD-1—both as single agents and as combination therapies with or without concurrent chemoimmunotherapy. Furthermore, we will review the role of bispecific T-cell engagers, anti-CD19 chimeric antigen receptor T-cell therapies, and hematopoietic stem cell transplantation in RT. To guide therapeutic decision-making, we will outline an algorithmic approach to the management of RT, with particular emphasis on prioritization of clinical trial enrollment and utilization of an ever-evolving array of novel therapies.
里氏转化（RT）是慢性淋巴细胞白血病/小淋巴细胞淋巴瘤（CLL）中一种罕见但极具破坏性的并发症。RT被定义为在CLL背景下发生的侵袭性淋巴瘤，通常为弥漫性大B细胞淋巴瘤。大多数RT病例中可检测到与先前CLL克隆的克隆关联，这种关联导致更侵袭性的临床病理动力学、对标准化学免疫治疗方案耐药以及更差的生存预后。综上所述，这些因素凸显了当前对改善RT患者预后的新型治疗策略存在重大未满足需求。通过本综述，我们将探讨针对BTK、BCL-2、CD79、CD20、PI3K和PD-1等靶点的新兴治疗方案（包括单药治疗及联合化疗免疫治疗方案）的最新数据。此外，我们将回顾双特异性T细胞衔接器、抗CD19嵌合抗原受体T细胞疗法以及造血干细胞移植在RT治疗中的作用。为指导临床决策，我们将概述RT管理的算法化路径，特别强调临床试验参与的优先顺序及不断演进的新型疗法体系的应用。
肿瘤(癌症)患者之家