{"entity":{"id":"lymphoma-bio-transformation","kind":"term","name":"Transformation: when a slow lymphoma turns into a fast one","aka":["Histological transformation","Transformed follicular lymphoma","Richter transformation","Richter syndrome"],"tldr":"An indolent lymphoma can change into an aggressive one, usually by acquiring new genetic faults in the same clone. It is the commonest reason a person who has been well for years becomes unwell quickly, and it is treated as the aggressive disease rather than the original one.","summary":"Transformation is a change in the behaviour of a clone, not the arrival of a second cancer. A follicular lymphoma that has been watched for years can acquire MYC rearrangement, TP53 loss or CDKN2A deletion and start behaving as a diffuse large B-cell lymphoma. Chronic lymphocytic leukaemia can do the same, and that version has its own name, Richter transformation.\n\nThe genetics of Richter transformation were read across 86 pathologically proven cases: TP53 disruption in 47.1% and MYC abnormality in 26.2% were the dominant lesions, while the usual drivers of de novo diffuse large B-cell lymphoma were rare or absent. Whether the large-cell clone is related to the leukaemic clone matters more than any drug does: clonally unrelated cases had median survival of 62.5 months against 14.2 months for related ones, and less TP53 disruption, 23.1% against 60.0% (Rossi 2011).\n\nWhat prompts the suspicion: a single node or site growing much faster than the rest, new B symptoms, a rising LDH, or a PET scan with one area far brighter than the others. What settles it is a biopsy of the brightest area, because the question is answered by tissue and nothing else answers it.\n\nWhat changes: treatment moves to an aggressive-lymphoma regimen, the clonal relationship is worth establishing because it changes the expected course, and a trial is often the right answer in Richter transformation, where outcomes with standard chemoimmunotherapy are poor.","asOf":"2026-09-30","links":[{"label":"Rossi et al., Blood 2011: the genetics of Richter syndrome in 86 pathologically proven cases","url":"https://doi.org/10.1182/blood-2010-09-302174"},{"label":"Horn et al., Blood 2013: MYC, BCL2 and BCL6 rearrangement and expression in 442 RICOVER patients","url":"https://doi.org/10.1182/blood-2012-06-435842"}],"tags":[],"related":[],"cancers":["follicular-lymphoma","richter-transformation-cll","marginal-zone-lymphoma","waldenstrom","non-hodgkin-lymphoma"],"sections":[],"technologies":["fdg-pet","cytogenetics-fish"],"targets":["myc-gene","tp53","cdkn2a"],"drugs":[],"companies":[],"institutions":[],"pathways":["clonal-evolution","p53-mdm2-axis"],"terms":["flipi","lymphoma-tx-pod24","deauville"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"category":"Cancer biology"},"route":"/terms/lymphoma-bio-transformation/","neighbours":{"cancer":[{"id":"follicular-lymphoma","kind":"cancer","name":"Follicular lymphoma","route":"/cancers/follicular-lymphoma/"},{"id":"marginal-zone-lymphoma","kind":"cancer","name":"Marginal zone lymphoma","route":"/cancers/marginal-zone-lymphoma/"},{"id":"non-hodgkin-lymphoma","kind":"cancer","name":"Non-Hodgkin lymphoma (all types)","route":"/cancers/non-hodgkin-lymphoma/"},{"id":"richter-transformation-cll","kind":"cancer","name":"Richter transformation of chronic lymphocytic leukaemia","route":"/cancers/richter-transformation-cll/"},{"id":"waldenstrom","kind":"cancer","name":"Waldenström macroglobulinaemia","route":"/cancers/waldenstrom/"}],"technology":[{"id":"cytogenetics-fish","kind":"technology","name":"Cytogenetics and FISH","route":"/technologies/cytogenetics-fish/"},{"id":"fdg-pet","kind":"technology","name":"FDG PET","route":"/technologies/fdg-pet/"}],"target":[{"id":"cdkn2a","kind":"target","name":"CDKN2A","route":"/targets/cdkn2a/"},{"id":"myc-gene","kind":"target","name":"MYC","route":"/targets/myc-gene/"},{"id":"tp53","kind":"target","name":"TP53","route":"/targets/tp53/"}],"pathway":[{"id":"clonal-evolution","kind":"pathway","name":"Clonal evolution & minimal residual disease","route":"/pathways/clonal-evolution/"},{"id":"p53-mdm2-axis","kind":"pathway","name":"The p53 network (guardian of the genome)","route":"/pathways/p53-mdm2-axis/"}],"term":[{"id":"deauville","kind":"term","name":"Deauville score and PET-adapted therapy","route":"/terms/deauville/"},{"id":"flipi","kind":"term","name":"FLIPI, FLIPI2 and POD24 (follicular lymphoma risk)","route":"/terms/flipi/"},{"id":"lymphoma-tx-pod24","kind":"term","name":"POD24: progression of follicular lymphoma within two years, and why it changes the plan","route":"/terms/lymphoma-tx-pod24/"}]}}