# Biology and treatment of Richter syndrome

Source: https://onco.cc/key-papers/paper-rossi-richter-syndrome-blood-2018/  
OnCo record `paper-rossi-richter-syndrome-blood-2018` (Key paper). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## Fields

- Kind: Key paper
- Last checked: 2026-09-17
- Journal: Blood
- Year: 2018
- DOI: 10.1182/blood-2018-01-791376
- Authors: Rossi D, Spina V, Gaidano G.
- What it means: 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.

## Sources

- Blood 2018: https://doi.org/10.1182/blood-2018-01-791376
- PubMed: https://pubmed.ncbi.nlm.nih.gov/29692342/

## Connected records

- cancers: [Richter transformation of chronic lymphocytic leukaemia](https://onco.cc/cancers/richter-transformation-cll/)
- journals: [Blood](https://onco.cc/journals/blood/)

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