文章：

里氏综合征治疗进展，包括新型联合疗法

Updates on the Treatment of Richter’s Syndrome, Including Novel Combination Approaches

原文发布日期：11 March 2025

DOI: 10.3390/cancers17060943

类型: Article

开放获取: 是

英文摘要：

Richter’s syndrome (RS) or transformation of chronic lymphocytic leukemia (CLL) into a more aggressive lymphoma (e.g., diffuse large B cell lymphoma, DLBCL) is a distinct disease that portends an overall poor prognosis and remains a challenge for clinicians to identify and treat effectively. This review of the current literature focuses on the pathology, diagnosis, and management of Richter’s syndrome. Clonally related RS has been found to have a worse prognosis than unrelated disease and the genomic profile of DLBCL-RS differs from that of de novo DLBCL. The standard of care therapy for RS has historically been chemoimmunotherapy; consolidative stem cell transplants have a role in improving durability of disease response. Given generally poor response rates to chemotherapy, there have been recent investigations into combination treatments with immune checkpoint inhibitors and small molecule targeted therapies, which have had mixed results. Additional studies are evaluating the use of bispecific antibodies, chimeric antigen receptor T cell therapy, and antibody drug conjugates. RS remains difficult to manage; however, advancements in the understanding of the underlying pathology of transformation and continued investigations into new therapies demonstrate promise for the future.

摘要翻译： 

里氏综合征（RS），即慢性淋巴细胞白血病（CLL）向更具侵袭性的淋巴瘤（如弥漫性大B细胞淋巴瘤，DLBCL）的转化，是一种预后普遍不良的独特疾病，其有效识别与治疗仍是临床医生面临的挑战。本文综述当前文献，重点探讨里氏综合征的病理学、诊断及临床管理。研究发现，克隆相关的RS预后较非相关疾病更差，且DLBCL-RS的基因组特征与原发性DLBCL存在差异。历史上，RS的标准治疗方案为化学免疫疗法；巩固性干细胞移植在提升疾病缓解持久性方面具有一定作用。鉴于化疗总体缓解率较低，近期研究探索了免疫检查点抑制剂与小分子靶向药物的联合疗法，但疗效结果不一。更多研究正在评估双特异性抗体、嵌合抗原受体T细胞疗法及抗体药物偶联物的应用。尽管RS的治疗仍具挑战，但对转化潜在病理机制的深入理解以及新型疗法的持续探索，为未来治疗带来了希望。

原文链接：

Updates on the Treatment of Richter’s Syndrome, Including Novel Combination Approaches