Contemporary Standard of Care Therapy for Richter's Transformation and Future Directions

Richter's transformation (RT) is a life-threatening evolution of chronic lymphocytic leukemia (CLL) into a more aggressive lymphoma, typically diffuse large B-cell lymphoma (DLBCL), marking a challenging juncture in CLL management due to the associated poor prognosis and limited treatment options. This review delves into the current therapeutic landscape for RT. Despite the modest efficacy of traditional chemoimmunotherapy (CIT) regimens such as R-CHOP and its variations, this regimen remains the most commonly recommended standard of care. Multiple therapeutic strategies are under investigation, including targeted kinase inhibitors, checkpoint inhibitors, bispecific antibodies, and CAR T therapy. Given the complex nature of RT and the evolving therapeutic paradigms, ongoing research is imperative to refine treatment strategies and integrate novel therapeutic agents to enhance survival and quality of life for people with RT. Given the lack of a clear standard of approach in the management of RT, patients with RT should be prioritized to enroll on clinical trials where feasible.