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

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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]
Copyright of Cancers is the property of MDPI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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  Data: Updates on the Treatment of Richter's Syndrome, Including Novel Combination Approaches.
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  Data: <searchLink fieldCode="AR" term="%22Jain%2C+Tanim%22">Jain, Tanim</searchLink><relatesTo>1</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Heyman%2C+Benjamin%22">Heyman, Benjamin</searchLink><relatesTo>2</relatesTo> (AUTHOR)<i> bheyman@health.ucsd.edu</i>
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  Data: <searchLink fieldCode="JN" term="%22Cancers%22">Cancers</searchLink>. Mar2025, Vol. 17 Issue 6, p943. 17p.
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  Data: *<searchLink fieldCode="DE" term="%22CHRONIC+lymphocytic+leukemia%22">CHRONIC lymphocytic leukemia</searchLink><br />*<searchLink fieldCode="DE" term="%22PREDICTIVE+tests%22">PREDICTIVE tests</searchLink><br />*<searchLink fieldCode="DE" term="%22HEMATOPOIETIC+stem+cell+transplantation%22">HEMATOPOIETIC stem cell transplantation</searchLink><br />*<searchLink fieldCode="DE" term="%22IMMUNOTHERAPY%22">IMMUNOTHERAPY</searchLink><br />*<searchLink fieldCode="DE" term="%22POSITRON+emission+tomography+computed+tomography%22">POSITRON emission tomography computed tomography</searchLink><br />*<searchLink fieldCode="DE" term="%22CELLULAR+therapy%22">CELLULAR therapy</searchLink><br />*<searchLink fieldCode="DE" term="%22CANCER+chemotherapy%22">CANCER chemotherapy</searchLink><br />*<searchLink fieldCode="DE" term="%22IMMUNE+checkpoint+inhibitors%22">IMMUNE checkpoint inhibitors</searchLink><br />*<searchLink fieldCode="DE" term="%22RICHTER+syndrome%22">RICHTER syndrome</searchLink><br />*<searchLink fieldCode="DE" term="%22SENSITIVITY+%26+specificity+%28Statistics%29%22">SENSITIVITY & specificity (Statistics)</searchLink><br />*<searchLink fieldCode="DE" term="%22DISEASE+risk+factors%22">DISEASE risk factors</searchLink><br />*<searchLink fieldCode="DE" term="%22SYMPTOMS%22">SYMPTOMS</searchLink>
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: 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]
– Name: AbstractSuppliedCopyright
  Label:
  Group: Ab
  Data: <i>Copyright of Cancers is the property of MDPI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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      – Type: doi
        Value: 10.3390/cancers17060943
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      – Code: eng
        Text: English
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        PageCount: 17
        StartPage: 943
    Subjects:
      – SubjectFull: CHRONIC lymphocytic leukemia
        Type: general
      – SubjectFull: PREDICTIVE tests
        Type: general
      – SubjectFull: HEMATOPOIETIC stem cell transplantation
        Type: general
      – SubjectFull: IMMUNOTHERAPY
        Type: general
      – SubjectFull: POSITRON emission tomography computed tomography
        Type: general
      – SubjectFull: CELLULAR therapy
        Type: general
      – SubjectFull: CANCER chemotherapy
        Type: general
      – SubjectFull: IMMUNE checkpoint inhibitors
        Type: general
      – SubjectFull: RICHTER syndrome
        Type: general
      – SubjectFull: SENSITIVITY & specificity (Statistics)
        Type: general
      – SubjectFull: DISEASE risk factors
        Type: general
      – SubjectFull: SYMPTOMS
        Type: general
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      – TitleFull: Updates on the Treatment of Richter's Syndrome, Including Novel Combination Approaches.
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              Text: Mar2025
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              Y: 2025
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