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Richter's transformation in patients with chronic lymphocytic leukaemia: a Nationwide Epidemiological Study

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Richter's transformation (RT) refers to the development of an aggressive lymphoma in patients with chronic lymphocytic leukemia (CLL). Approximately, 2-10% of patients with CLL develop RT, most often as diffuse large B-cell lymphoma. To assess the incidence of RT, we examined risk factors for RT and death among patients with RT in a nationwide CLL cohort (from 2008 to 2016). Among 3772 patients, 113 had biopsy-proven RT. With a median follow-up of 4.3 years, the 5-year cumulative incidence of RT was 2.8%. Advanced Binet stage (B/C) (p<.001), unmutated IGHV (p<.001), and del(17p) (p<.001) were independently associated with risk of developing RT. Half of the patients with RT (49%) were treatment-naïve prior to transformation and demonstrated longer survival after RT compared to patients previously treated for CLL (6.1 vs. 2.8 years, p=.03). Whether this finding could be explained by a higher proportion of clonally unrelated RT among treatment-naïve patients, remain to be addressed.

Original languageEnglish
JournalLeukemia and Lymphoma
Issue number6
Pages (from-to)1435-1444
Number of pages10
Publication statusPublished - Jun 2020

    Research areas

  • Chronic lymphocytic leukemia, diffuse large B-cell lymphoma, Hodgkin lymphoma, prognostication, Richter’s transformation

ID: 60920959