A new nationwide study showed that the 5-year cumulative incidence of Richter transformation (RT) among patients with chronic lymphocytic leukemia (CLL) was 2.8%.1
Of those patients whose disease transformed, about half (49%) were treatment-naive prior to transformation.
The researchers identified 3 factors associated with increased risk for transformation: advanced Binet stage (P<.001), unmutated immunoglobulin heavy chain variable IGHV genes (P <.001), and del(17p) (P <.001).
“These three prognostic factors have previously been reported as risk factors for RT development and are also included in the international prognostic index for patients with CLL (CLL-IPI), reflecting similar risk factors for CLL progression and RT,” the researchers wrote. “Nevertheless, as the treatment options for patients with progressive CLL have improved, resulting in improved survival for patients with CLL over the last decades, RT becomes an even more important unmet challenge for patients with the most aggressive CLL phenotype.”
Among those patients diagnosed with RT after initiating treatment for CLL, 53% developed it after first-line treatment, 24% after second-line treatment, and 22% after 3 or more lines.
The median overall survival among patients with CLL and RT was 3.1 years. Those patients who were previously treated for CLL had a worse overall survival than those who were treatment naive (2.8 years vs 6.1 years; P =.03).
The epidemiological study looked at 3772 patients with CLL from a nationwide cohort diagnosed between 2008 and 2016. Patients were followed for a median of 4.3 years.
“Knowledge of incidence, risk factors and prognostic markers in a population-based cohort is therefore essential for improving patient management,” researchers wrote.
Disclosure: Some of the authors disclosed financial relationships with pharmaceutical companies and medical device manufacturers. For a full list of disclosures, please refer to the original study.
Ben-Dali Y, Hleuhel MH, da Cunha-Bang C, et al. Richter’s transformation in patients with chronic lymphocytic leukaemia: a nationwide epidemiological study [published online February 7, 2020]. Leuk Lymphoma. doi :10.1080/10428194.2020.1719092