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

Yasmin Ben-Dali, Mariam H Hleuhel, Caspar da Cunha-Bang, Christian Brieghel, Christian B Poulsen, Erik Clasen-Linde, Hans H N Bentzen, Henrik Frederiksen, Ilse Christiansen, Linda H Nielsen, Lisbeth Enggaard, Marie Helleberg, Michael Clausen, Mikael Frederiksen, Robert S Pedersen, Carsten U Niemann, Michael A Andersen

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Abstrakt

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.

OriginalsprogEngelsk
TidsskriftLeukemia and Lymphoma
Vol/bind61
Udgave nummer6
Sider (fra-til)1435-1444
Antal sider10
ISSN1042-8194
DOI
StatusE-pub ahead of print - 7. feb. 2020

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  • Citationsformater

    Ben-Dali, Y., Hleuhel, M. H., da Cunha-Bang, C., Brieghel, C., Poulsen, C. B., Clasen-Linde, E., Bentzen, H. H. N., Frederiksen, H., Christiansen, I., Nielsen, L. H., Enggaard, L., Helleberg, M., Clausen, M., Frederiksen, M., Pedersen, R. S., Niemann, C. U., & Andersen, M. A. (2020). Richter's transformation in patients with chronic lymphocytic leukaemia: a Nationwide Epidemiological Study. Leukemia and Lymphoma, 61(6), 1435-1444. https://doi.org/10.1080/10428194.2020.1719092