Immunochemotherapy for Richter syndrome: current insights

Abstract

Bartosz Puła,1 Aleksander Salomon-Perzyński,1 Monika Prochorec-Sobieszek,2,3 Krzysztof Jamroziak1 1Department of Hematology, Institute of Hematology and Transfusion Medicine, Warsaw, Poland; 2Department of Diagnostic Hematology, Institute of Hematology and Transfusion Medicine, Warsaw, Poland; 3Department of Pathology and Laboratory Medicine, Maria Sklodowska-Curie Institute – Oncology Center, Warsaw, Poland Abstract: Richter syndrome (RS) is recognized as the development of a secondary and aggressive lymphoma during the clinical course of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). Most of such histological transformations are from RS to diffuse large B-cell lymphoma (DLBCL-RS, 90%) and Hodgkin’s lymphoma (HL-RS, 10%). ­Histopathological examination is a prerequisite for diagnosis. It is crucial to assess the relationship between the RS clone and the underlying CLL/SLL because clonally related DLBCL-RS has a poor outcome, while clonally unrelated cases have a prognosis similar to de novo DLBCL. An anti-CD20 antibody-based immunochemotherapy is hitherto the frontline treatment of choice for DLBCL-RS; nonetheless, the results are unsatisfactory. Allogeneic stem cell transplantation should be offered to younger and fit patients as a consolidative treatment; however, the majority of the patients may not be qualified for this procedure. The HL-RS transformation has better outcomes than those of DLBCL-RS and can effectively be treated by the adriamycin, bleomycin, vinblastine, and dacarbazine regimen. Although novel agents are currently being investigated for RS, immunochemotherapy nevertheless remains a standard treatment for DLBCL-RS. Keywords: Richter syndrome, Richter transformation, chronic lymphocytic leukemia, diffuse large B-cell lymphoma, Hodgkin’s lymphoma

Keywords

• Richter syndrome
• Richter transformation
• chronic lymphocytic leukemia
• diffuse large B-cell lymphoma
• Hodgkin lymphoma