{"entity":{"id":"richter-transformation","kind":"term","name":"Richter transformation","aka":[],"tldr":"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.","asOf":"2026-09-07","wikipedia":"https://en.wikipedia.org/wiki/Richter%27s_transformation","links":[{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Richter%27s_transformation"}],"tags":[],"related":[],"cancers":["cll","dlbcl"],"sections":[],"technologies":["checkpoint-inhibitor","t-cell-engager","allogeneic-hsct"],"targets":[],"drugs":["pirtobrutinib","zanubrutinib","glofitamab"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"category":"Clinical"},"route":"/terms/richter-transformation/","neighbours":{"cancer":[{"id":"cll","kind":"cancer","name":"Chronic lymphocytic leukaemia","route":"/cancers/cll/"},{"id":"dlbcl","kind":"cancer","name":"Diffuse large B-cell lymphoma","route":"/cancers/dlbcl/"},{"id":"cll-relapsed","kind":"cancer","name":"Relapsed or refractory chronic lymphocytic leukaemia","route":"/cancers/cll-relapsed/"},{"id":"richter-transformation-cll","kind":"cancer","name":"Richter transformation of chronic lymphocytic leukaemia","route":"/cancers/richter-transformation-cll/"}],"technology":[{"id":"allogeneic-hsct","kind":"technology","name":"Allogeneic stem cell transplantation","route":"/technologies/allogeneic-hsct/"},{"id":"checkpoint-inhibitor","kind":"technology","name":"Immune checkpoint inhibitors","route":"/technologies/checkpoint-inhibitor/"},{"id":"t-cell-engager","kind":"technology","name":"T-cell engagers (bispecific)","route":"/technologies/t-cell-engager/"}],"drug":[{"id":"glofitamab","kind":"drug","name":"Glofitamab","route":"/drugs/glofitamab/"},{"id":"pirtobrutinib","kind":"drug","name":"Pirtobrutinib","route":"/drugs/pirtobrutinib/"},{"id":"zanubrutinib","kind":"drug","name":"Zanubrutinib","route":"/drugs/zanubrutinib/"}],"pathway":[{"id":"lineage-plasticity-neuroendocrine","kind":"pathway","name":"Lineage plasticity & neuroendocrine transformation","route":"/pathways/lineage-plasticity-neuroendocrine/"}]}}