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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.
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.
The 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).
What 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.
What 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.
In plain words · MYC (Myc proto-oncogene protein) is a protein that switches other genes on and off. The public catalogues list it as a drug target, an oncogene driver, a tumour suppressor, a biomarker and a fusion partner, and clinical evidence ties its variants to diagnosis, prognosis or drug response. Tied to Non-Hodgkin lymphoma, Skin cancer, Multiple myeloma and 5 more.
Showing the target this term concerns: MYC.
Shares POD24: progression of follicular lymphoma within two years, and why it changes the plan, FLIPI, FLIPI2 and POD24 (follicular lymphoma risk), Waldenström macroglobulinaemia, Marginal zone lymphoma.
Shares Richter transformation of chronic lymphocytic leukaemia, Marginal zone lymphoma, Follicular lymphoma, Non-Hodgkin lymphoma (all types).
Shares FLIPI, FLIPI2 and POD24 (follicular lymphoma risk), Waldenström macroglobulinaemia, Marginal zone lymphoma, Follicular lymphoma.
Shares Richter transformation of chronic lymphocytic leukaemia, Clonal evolution & minimal residual disease, Marginal zone lymphoma, Follicular lymphoma.
Shares Waldenström macroglobulinaemia, Marginal zone lymphoma, Follicular lymphoma, Non-Hodgkin lymphoma (all types).
Shares POD24: progression of follicular lymphoma within two years, and why it changes the plan, FLIPI, FLIPI2 and POD24 (follicular lymphoma risk), Waldenström macroglobulinaemia, Marginal zone lymphoma.
Shares Richter transformation of chronic lymphocytic leukaemia, Cytogenetics and FISH, MYC, Follicular lymphoma.
Shares Deauville score and PET-adapted therapy, Waldenström macroglobulinaemia, Marginal zone lymphoma, Follicular lymphoma.