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Richter's transformation in patients with chronic lymphocytic leukaemia: a Nationwide Epidemiological Study. 2020
Yasmin Ben-Dali, and
Mariam H Hleuhel, and
Caspar da Cunha-Bang, and
Christian Brieghel, and
Christian B Poulsen, and
Erik Clasen-Linde, and
Hans H N Bentzen, and
Henrik Frederiksen, and
Ilse Christiansen, and
Linda H Nielsen, and
Lisbeth Enggaard, and
Marie Helleberg, and
Michael Clausen, and
Mikael Frederiksen, and
Robert S Pedersen, and
Carsten U Niemann, and
Michael A Andersen
Department of Haematology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.
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.
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