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

被引:0
作者
Jain, Tanim [1 ]
Heyman, Benjamin [2 ]
机构
[1] Univ Calif San Diego, Dept Med, Div Hematol Oncol, La Jolla, CA 92093 USA
[2] Univ Calif San Diego, UC San Diego Moores Canc Ctr, Dept Med, Div Regenerat Med, La Jolla, CA 92093 USA
关键词
Richter's syndrome; diffuse large B cell lymphoma; chronic lymphocytic leukemia; CHRONIC-LYMPHOCYTIC-LEUKEMIA; B-CELL LYMPHOMA; FRACTIONATED CYCLOPHOSPHAMIDE; LIPOSOMAL DAUNORUBICIN; TRANSFORMATION RT; CLINICAL-TRIAL; RISK-FACTORS; FOLLOW-UP; PHASE-II; CLL;
D O I
10.3390/cancers17060943
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
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.
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页数:17
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