Biology and treatment of Richter syndrome
This review lays out how chronic lymphocytic leukaemia transforms into aggressive lymphoma, which genetic changes drive it, and how the diffuse large B-cell type should be treated, including the role of transplant and new agents.
Overview
Comprehensive review of Richter syndrome covering incidence, clonal relationship to the underlying CLL, the roles of TP53, NOTCH1, MYC and CDKN2A lesions, the effect of BTK inhibitor exposure, diagnostic work-up with PET-directed biopsy, prognostic scores, chemoimmunotherapy outcomes, consolidation with stem cell transplantation, and emerging targeted and immune therapies.
The page's approach to Richter transformation (biopsy the hottest node, R-CHOP-type therapy, consolidate with transplant if fit, trial enrolment otherwise) follows the framework in this review.
- Predates the bispecific antibody and pirtobrutinib data that now shape trials.