{"entity":{"id":"paper-rossi-richter-syndrome-blood-2018","kind":"paper","name":"Biology and treatment of Richter syndrome","aka":[],"tldr":"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.","summary":"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.","asOf":"2026-09-17","links":[{"label":"Blood 2018","url":"https://doi.org/10.1182/blood-2018-01-791376"},{"label":"PubMed","url":"https://pubmed.ncbi.nlm.nih.gov/29692342/"}],"tags":[],"related":[],"cancers":["richter-transformation-cll"],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":["blood"],"dependsOn":[],"notes":[],"journal":"Blood","year":2018,"doi":"10.1182/blood-2018-01-791376","pmid":"29692342","authors":"Rossi D, Spina V, Gaidano G.","paperType":"review","findings":[],"whatItMeans":"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.","caveats":["Predates the bispecific antibody and pirtobrutinib data that now shape trials."],"changedPractice":false},"route":"/key-papers/paper-rossi-richter-syndrome-blood-2018/","neighbours":{"cancer":[{"id":"richter-transformation-cll","kind":"cancer","name":"Richter transformation of chronic lymphocytic leukaemia","route":"/cancers/richter-transformation-cll/"}],"journal":[{"id":"blood","kind":"journal","name":"Blood","route":"/journals/blood/"}]}}