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Immunochemotherapy for Richter syndrome: current insights
Bartosz Puła1, Aleksander Salomon-Perzyński1, Monika Prochorec-Sobieszek2,3
1Department of Hematology, Institute of Hematology and Transfusion Medicine, Warsaw, Poland, k.m.jamroziak@gmail.com.
Immunotargets and Therapy
|February 22, 2019
Summary
Richter syndrome (RS) is an aggressive lymphoma developing from chronic lymphocytic leukemia (CLL). Diagnosis requires histopathology, and treatment varies based on subtype, with limited success for diffuse large B-cell lymphoma-RS.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Richter syndrome (RS) is a transformation from chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) into aggressive lymphoma.
- The majority of transformations are to diffuse large B-cell lymphoma (DLBCL-RS, 90%), with a smaller proportion to Hodgkin's lymphoma (HL-RS, 10%).
Purpose of the Study:
- To highlight the diagnostic criteria and prognostic implications of Richter syndrome.
- To review current treatment strategies and outcomes for DLBCL-RS and HL-RS.
Main Methods:
- Diagnosis relies on histopathological examination.
- Assessment of the clonal relationship between RS and underlying CLL/SLL is critical for prognosis.
Main Results:
- Clonally related DLBCL-RS portends a poor prognosis, unlike clonally unrelated cases which resemble de novo DLBCL.
- Anti-CD20 antibody-based immunochemotherapy is the standard frontline treatment for DLBCL-RS but yields unsatisfactory results.
- Allogeneic stem cell transplantation is an option for select younger patients.
- HL-RS demonstrates better outcomes and responds well to the ABVD regimen (adriamycin, bleomycin, vinblastine, dacarbazine).
Conclusions:
- Accurate histopathological diagnosis and clonal relationship assessment are vital for managing Richter syndrome.
- Current treatments for DLBCL-RS are suboptimal, necessitating further research into novel agents.
- HL-RS has a more favorable prognosis and established treatment protocols.
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