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Two Decades of Data Confirm Poor Prognosis With Richter Transformation in CLL

By: Celeste L. Dixon
Posted: Monday, July 20, 2020

Confirming the poor prognosis and high mortality associated with Richter transformation in patients with chronic lymphocytic leukemia (CLL) are retrospective analysis results published in Leukemia. The data were pooled from 2,975 patients with advanced CLL enrolled in the German CLL Study Group who underwent front-line treatment in eight clinical trials between 1999 and 2016; they were observed for a median of 53 months.

Of that group, 103 patients (3%) developed Richter transformation, characterized by aggressive lymphoma, after receiving first-line chemotherapy or chemoimmunotherapy. This figure was in line with the reported incidence range, 2% to 10%, of Richter transformation among patients with CLL, reported Othman Al-Sawaf, MD, of the University of Cologne, Germany, and colleagues. Almost all with Richter transformation were diagnosed with diffuse large B-cell lymphoma (DLBCL); eight patients had Hodgkin lymphoma.

From the time of their initial CLL diagnosis, the median overall survival for patients without Richter transformation was 167 months, whereas it was 71 months for those with Richter transformation (hazard ratio = 2.64). About half of the patients with Richter transformation (n = 47) received CHOP-like regimens (cyclophosphamide, doxorubicin, vincristine, prednisone), reported the team, and several subsequently underwent allogeneic (n = 3) and autologous (n = 2) stem cell transplantation. However, the median overall survival after diagnosis of Richter transformation was 9 months.

No established standard of care exists for patients who develop Richter transformation. “Most patients are treated comparably to de novo DLBCL patients with chemoimmunotherapies,” stated Dr. Al-Sawaf and co-investigators. However, “while this regimen achieves high response rates in de novo DLBCL and even cures up to 80% of patients, patients with Richter transformation are rarely cured by chemoimmunotherapy.”

Disclosure: The study authors’ disclosure information can be found at nature.com.



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