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Richter Transformation of Chronic Lymphocytic Leukemia—Are We Making Progress?

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Abstract   

Purpose of Review

The treatment paradigm of chronic lymphocytic leukemia (CLL) has dramatically changed with the advent of novel targeted agents over the past decade. Richter transformation (RT), or the development of an aggressive lymphoma from a background of CLL, is a well-recognized complication of CLL and carries significantly poor clinical outcomes. Here, we provide an update on current diagnostics, prognostication, and contemporary treatment of RT.

Recent Findings

Several genetic, biologic, and laboratory markers have been proposed as candidate risk factors for the development of RT. Although a diagnosis of RT is typically suspected based on clinical and laboratory findings, tissue biopsy is essential for histopathologic confirmation of diagnosis. The standard of care for RT treatment at this time remains chemoimmunotherapy with the goal of proceeding to allogeneic stem cell transplantation in eligible patients. Several newer treatment modalities are being studied for use in the management of RT, including small molecules, immunotherapy, bispecific antibodies, and chimeric antigen receptor T-cell (CAR-T) therapy.

Summary

The management of patients with RT remains a challenge. Ongoing trials show enormous promise for newer classes of therapy in RT, with the hope being that these agents can synergize, and perhaps supersede, the current standard of care in the near future.

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Data Availability

Requests regarding data availability not already shared in the supplemental material should be made to the corresponding author.

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Acknowledgements

The authors thank Dr. Michael Bold, MD (Department of Radiology, Mayo Clinic, Rochester, MN) for providing the radiology images of PET-CT scans in Fig. 2.

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  1. Division of Hematology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA

    Hadiyah Y. Audil & Sameer A. Parikh

  2. Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA

    Samuel R. Kosydar

  3. Division of Hematopathology, Mayo Clinic, Rochester, MN, USA

    Daniel P. Larson

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Research funding has been provided to the institution from Pharmacyclics, Janssen, AstraZeneca, TG Therapeutics, Merck, AbbVie, and Ascentage Pharma for clinical studies in which S.A.P. is a principal investigator. S.A.P. has received honoraria for participation in consulting activities/advisory board meetings for Pharmacyclics, Merck, AstraZeneca, Genentech, GlaxoSmithKline, Adaptive Biotechnologies, Amgen, and AbbVie (no personal compensation); and from DynaMed, Aptitude Health, Curio Science, and MedEd on the Go (with personal compensation). All other authors declare no conflict of interest.

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Audil, H.Y., Kosydar, S.R., Larson, D.P. et al. Richter Transformation of Chronic Lymphocytic Leukemia—Are We Making Progress?. Curr Hematol Malig Rep 18, 144–157 (2023). https://doi.org/10.1007/s11899-023-00701-y

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