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Incidence of Richter’s Transformation in the Targeted Therapy Era

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Medically reviewed by Dr. Brian Koffman

The Bottom Line:

While the incidence of Richter’s transformation has not changed dramatically with the introduction of targeted therapies, those who received CLL / SLL treatment in the targeted therapy era appear to have a lower risk of Richter’s transformation compared with those who received treatment when chemoimmunotherapy was the standard of care.

Who Performed the Research and Where Was it Presented:

Dr. Paul Hampel and colleagues from the Mayo Clinic presented the results at the American Society for Hematology (ASH) Annual Meeting 2023.

Background:

Richter’s transformation is a rare complication of chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL), where the disease turns into an aggressive lymphoma known as diffuse large B cell lymphoma (DLBCL). It has an extremely poor prognosis, and current treatments do not provide durable remissions. Previous studies have estimated that Richter’s transformation occurs in 1-5% of CLL patients. This study looked at the incidence of Richter’s transformation in the era of targeted therapy (2014 – present) compared with the era before targeted therapies (before 2014) when chemoimmunotherapy was the standard of care.

Methods and Participants:

This study included patients with previously untreated CLL / SLL who were seen at Mayo Clinic within 12 months of their diagnosis between 2000 and 2023. These patients were followed prospectively to see if they developed Richter’s transformation.

Results:

  • In total, 1981 patients were diagnosed with CLL / SLL in the pre-targeted therapy era, and 1366 patients were diagnosed in the targeted therapy era.
  • The proportion of patients who developed Richter’s transformation within one year of their CLL / SLL diagnosis was 0.6% in the pre-targeted therapy era and 0.5% in the targeted therapy era.
  • The proportion of patients who developed Richter’s transformation within five years of their CLL / SLL diagnosis was 2.1% in the pre-targeted therapy era and 1.1% in the targeted therapy era.
  • These numbers include patients who received treatment and those who did not need treatment (i.e., patients in the watch-and-wait phase).
  • When only patients who received CLL / SLL treatment were included, the proportion of patients who developed Richter’s transformation within five years of their diagnosis was 3.7% in the pre-targeted therapy era and 1.9% in the targeted therapy era.

Conclusions:

While there were some early concerns that the incidence of Richter’s transformation might be higher in the targeted therapy era due to patients living longer, this does not seem to be the case. In fact, it appears that the incidence of Richter’s transformation may actually be lower in patients who received CLL / SLL treatment in the targeted therapy era, which would be welcome news. However, further research is needed to confirm these findings.

Links and Resources:

Watch the interview on the abstract here:

Incidence of Richter’s Transformation in the Targeted Therapy Era – Dr. Paul Hampel ASH 2023

You can read the ASH abstract here: Incidence of Richter Transformation in Patients with Chronic Lymphocytic Leukemia (CLL) /Small Lymphocytic Lymphoma (SLL): A Cohort Study Evaluating Different Therapeutic Eras.

Take care of yourself first.

Ann Liu, PhD