# Richter transformation

Source: https://onco.cc/terms/richter-transformation/  
OnCo record `richter-transformation` (Term). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

When slow CLL suddenly turns into an aggressive lymphoma. Rare, hard to treat, and the focus of new immunotherapy trials.

## Summary

Occurs in 2-10% of CLL, usually as clonally related diffuse large B-cell lymphoma; median survival historically under a year. Chemoimmunotherapy is poor; PD-1 blockade with BTK inhibition (zanubrutinib-tislelizumab RT1 trial, ORR ~58%), pirtobrutinib, venetoclax-based combinations, CD20×CD3 bispecifics (epcoritamab, glofitamab), and CAR-T show activity. Allogeneic transplant for responders.

## Fields

- Kind: Term
- Last checked: 2026-09-07

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Richter%27s_transformation
- Wikipedia: https://en.wikipedia.org/wiki/Richter%27s_transformation

## Connected records

- cancers: [Chronic lymphocytic leukaemia](https://onco.cc/cancers/cll/), [Diffuse large B-cell lymphoma](https://onco.cc/cancers/dlbcl/), [Relapsed or refractory chronic lymphocytic leukaemia](https://onco.cc/cancers/cll-relapsed/), [Richter transformation of chronic lymphocytic leukaemia](https://onco.cc/cancers/richter-transformation-cll/)
- technologies: [Allogeneic stem cell transplantation](https://onco.cc/technologies/allogeneic-hsct/), [Immune checkpoint inhibitors](https://onco.cc/technologies/checkpoint-inhibitor/), [T-cell engagers (bispecific)](https://onco.cc/technologies/t-cell-engager/)
- drugs: [Glofitamab](https://onco.cc/drugs/glofitamab/), [Pirtobrutinib](https://onco.cc/drugs/pirtobrutinib/), [Zanubrutinib](https://onco.cc/drugs/zanubrutinib/)
- pathways: [Lineage plasticity & neuroendocrine transformation](https://onco.cc/pathways/lineage-plasticity-neuroendocrine/)

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JSON: https://onco.cc/api/v1/entities/richter-transformation.json