Bibliographic Details
| Title: |
Updates on the Treatment of Richter's Syndrome, Including Novel Combination Approaches. |
| Authors: |
Jain, Tanim1 (AUTHOR), Heyman, Benjamin2 (AUTHOR) bheyman@health.ucsd.edu |
| Source: |
Cancers. Mar2025, Vol. 17 Issue 6, p943. 17p. |
| Subject Terms: |
*CHRONIC lymphocytic leukemia, *PREDICTIVE tests, *HEMATOPOIETIC stem cell transplantation, *IMMUNOTHERAPY, *POSITRON emission tomography computed tomography, *CELLULAR therapy, *CANCER chemotherapy, *IMMUNE checkpoint inhibitors, *RICHTER syndrome, *SENSITIVITY & specificity (Statistics), *DISEASE risk factors, *SYMPTOMS |
| Abstract: |
Simple Summary: Chronic lymphocytic leukemia (CLL) is an indolent disease that can transform into more aggressive forms of lymphoma, termed Richter's syndrome or transformation. This disease can be difficult to differentiate from aggressive CLL in terms of diagnosis and has historically been challenging to treat due to poor responses to chemotherapy. However, there have been recent studies evaluating the use of immunotherapy and small molecule targeted therapies for Richter's syndrome, as single agents and in combination regimens, which have demonstrated some promise. This review aims to summarize the current understanding of the pathology, diagnosis, and treatment options for diffuse large B cell lymphoma-: Richter's syndrome. 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. [ABSTRACT FROM AUTHOR] |
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