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

Abstract

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.

Keywords:

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

Acknowledgements

The authors specially thank all coauthors who have supported furthering research experience through collaboration and feedback.

Statement of prior presentation: Presented in abstract form at the 60th annual meeting of the American Society of Hematology, San Diego, CA, 1 December 2018. Presented in oral presentation form at the annual meeting of the Danish Society of Hematology, Middelfart, DK, 16 March 2019.

Disclosure statement

CUN received funding and/or consultancy fees from Janssen, Roche, Abbvie, Acerta, Sunesis, AstraZeneca, and BSL Behring outside this study. CC, CB, CP, EC, HB, HF, IC, LN, LE, MH, MC, MF, RP, and MA declare no conflicts of interest.

Additional information

Funding

This research received support from the Research Committee, Rigshospitalet. This project was supported by research funding from Novo Nordisk Foundation (NNF Grant NNF16OC0019302), Danish National Research Foundation (DNRF Grant 126) and Rigshospitalet.