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EHA 2025 | Recommendations from the ERIC consensus on the diagnosis, staging, and treatment of RT
Alexey Danilov, MD, PhD, City of Hope, Duarte, CA, discusses the challenges of treating Richter’s transformation (RT) and the updated ERIC recommendations. He gives guidance on diagnosing and staging RT, and recommends that all patients should be enrolled in a clinical trial if possible. This interview took place at the 30th Congress of the European Hematology Association (EHA) in Milan, Italy.
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Transcript
Richter’s transformation remains an unmet medical need in the field of chronic lymphocytic leukemia. While it’s fairly rare, it’s very difficult to treat and unfortunately many patients die of Richter’s transformation. So this is certainly not a diagnosis one would like to receive and there are really no therapies approved specifically for Richter transformation or RT...
Richter’s transformation remains an unmet medical need in the field of chronic lymphocytic leukemia. While it’s fairly rare, it’s very difficult to treat and unfortunately many patients die of Richter’s transformation. So this is certainly not a diagnosis one would like to receive and there are really no therapies approved specifically for Richter transformation or RT. So that was the goal of ERIC Assembly to harmonize some of the guidelines on how to approach Richter transformation across the world. So Richter transformation typically presents with significant symptoms such as rapidly progressive lymphadenopathy, B symptoms, elevated LDH. So it is important to suspect RT when patients present with a multitude of debilitating symptoms. It is also important to diagnose Richter transformation on a biopsy. So when you suspect Richter transformation, a biopsy is essentially paramount. PET-CT aids in locating the biopsy site which is likely to give the highest yield because false-negative biopsy is unfortunately a problem. So one would want to biopsy a lesion with the highest SUV. Then as there are no good therapies for patients with RT, most patients should participate in a clinical trial if possible. And staging of Richter transformation there is a big discussion on that as well how to stage it on a clinical trial and it is the consensus that Lugano criteria would be preferred PET-based Lugano criteria to accurately assess efficacy of therapies on the course of Richter transformation given underlying, also presence of underlying CLL. Another small point to make which is sometimes difficult to execute is it is very important to test for clonal relatedness between RT and CLL. There is about maybe 15 or 20% of patients who actually have clonally unrelated diffuse large B-cell lymphoma. So it is not a true Richter’s transformation in that sense. These patients tend to do better with standard chemoimmunotherapy regimens, and it is really important to identify these patients. And meanwhile, patients with clonally-related CLL-RT should be treated with novel therapies, including bispecific antibodies, CAR T-cells, again, preferably on a clinical trial.
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