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Journal article

Richter syndrome

  • Condoluci, Adalgisa Division of Hematology, Oncology Institute of Southern Switzerland (IOSI), Bellinzona, Switzerland - Laboratory of Experimental Hematology, Institute of Oncology Research (IOR), Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland
  • Rossi, Davide Division of Hematology, Oncology Institute of Southern Switzerland (IOSI), Bellinzona, Switzerland - Laboratory of Experimental Hematology, Institute of Oncology Research (IOR), Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland
    2021
Published in:
  • Current Oncology Reports. - Springer. - 2021, vol. 23
Richter syndrome
CLL
Transformation
DLBCL
Hodgkin lymphoma
English Purpose of Review Richter syndrome (RS) is an uncommon but aggressive evolution of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). RS is an unmet clinical need in the field of CLL. Recent advances in understanding the biology of this condition provide the rationale for testing new therapeutic concepts in order to improve the outcome of patients developing RS, which is so far poor. In this review, we summarize disease characteristics and available therapeutic options for RS. Recent Findings Current regimens with novel agents in monotherapy have shown little impact on survival. Nevertheless, the better reported outcome for RS has been achieved with the combination of chemo-immunotherapy with a novel agent, confirming the synergistic effect of the approaches. Still, the frailty of this population may impose a less toxic management leaving most patients with no reasonable therapeutic option. Summary Treatment options for RS need to be further expanded. Preclinical models in current development may allow to explore actionable pathways and identify new drug targeted combinations.
Language
  • English
Classification
Medicine
License
CC BY
Open access status
hybrid
Identifiers
Persistent URL
https://n2t.net/ark:/12658/srd1319234
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