International consensus statement on diagnosis, evaluation, and research of Richter transformation: the ERIC recommendations
Richter transformation (RT) is defined as an aggressive lymphoma emerging in patients with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL). Despite novel therapeutics developed in CLL, RT is associated with poor outcomes. In light of recent progress regarding the diagnostic procedures and therapeutic concepts of RT, an international group of experts, under the coordination of the European Research Initiative on CLL, has developed consensus recommendations for clinical procedures and future research on this disease. Patients with RT typically present with a rapid clinical decline, worsening B-symptoms, elevated lactate dehydrogenase, and/or rapidly enlarging lymphadenopathy. Workup should include a positron emission tomography-computed tomography scan for patients with suspected RT. An excisional biopsy should be taken from an accessible lesion, preferably with the highest fluorodeoxyglucose avidity, and analyzed for the presence of aggressive lymphoma. The molecular relationship to the original CLL clone(s) should be defined. Because no effective standard treatment for RT exists, patients should be treated in a clinical trial. Response of both RT and CLL should be assessed at an early time point, and survival end points should be prioritized in trial design. We hope that these recommendations can help to harmonize clinical and translational research and improve outcomes for patients with RT.
Authors
- Tanya Siddiqi (ORCID: https://orcid.org/0000-0001-5292-8298)
- Jennifer Ann Woyach (ORCID: https://orcid.org/0000-0002-3403-9144)
- Rebecca L. King (ORCID: https://orcid.org/0000-0002-7132-0226)
- Maurilio Ponzoni (ORCID: https://orcid.org/0000-0002-0055-325X)
- José I. Martı́n-Subero (ORCID: https://orcid.org/0000-0001-8809-5195)
- Stephan Stilgenbauer (ORCID: https://orcid.org/0000-0002-6830-9296)
- Constantine Si Lun Tam (ORCID: https://orcid.org/0000-0002-9759-5017)
- Francesca Maria Rossi (ORCID: https://orcid.org/0000-0001-8438-1661)
- Elisa ten Hacken (ORCID: https://orcid.org/0000-0003-1573-8799)
- Paul Joseph Hampel (ORCID: https://orcid.org/0000-0003-1292-3024)
- Gianluca Gaïdano (ORCID: https://orcid.org/0000-0002-4681-0151)
- Erin Michelle Parry (ORCID: https://orcid.org/0000-0002-3382-9769)
- Ohad Benjamini (ORCID: https://orcid.org/0000-0003-1123-3339)
- Toby A. Eyre (ORCID: https://orcid.org/0000-0002-6631-9749)
- Carolyn Owen (ORCID: https://orcid.org/0000-0003-4110-1708)
- Rodney J. Hicks (ORCID: https://orcid.org/0000-0002-0758-0824)
- Matthew Steven Davids (ORCID: https://orcid.org/0000-0003-4529-2003)
- Arnon Philip Kater (ORCID: https://orcid.org/0000-0003-3190-1891)
- Alexey Valeryevich Danilov (ORCID: https://orcid.org/0000-0003-4461-0970)
- Anna Maria Frustaci (ORCID: https://orcid.org/0000-0003-2587-7901)
- Adam S. Kittai (ORCID: https://orcid.org/0000-0002-4891-6144)
- Michael J. Hallek (ORCID: https://orcid.org/0000-0002-7425-4455)
- Monia Marchetti (ORCID: https://orcid.org/0000-0001-7615-0572)
- Philip A. Thompson (ORCID: https://orcid.org/0000-0003-2086-6031)
- Othman Al‐Sawaf (ORCID: https://orcid.org/0000-0001-9895-0570)
- Paolo Ghia (ORCID: https://orcid.org/0000-0003-3750-7342)
- Yair Herishanu
- William G. Wierda
- Barbara Eichhorst
Institutions
- Institució Catalana de Recerca i Estudis Avançats (ES)
- Università degli Studi del Piemonte Orientale “Amedeo Avogadro” (IT)
- City Of Hope National Medical Center (US)
- The Royal Melbourne Hospital (AU)
- Mayo Clinic (US)
- The University of Texas MD Anderson Cancer Center (US)
- Vita-Salute San Raffaele University (IT)
- Tel Aviv University (IL)
- University of Calgary (CA)
- University of Cologne (DE)
- Universität Ulm (DE)
- Cornell University (US)
- Tel Aviv Sourasky Medical Center (IL)
- Sheba Medical Center (IL)
- Peter MacCallum Cancer Centre (AU)
- Churchill Hospital (GB)
- The Alfred Hospital (AU)
- WinnMed (US)
- St Vincent's Hospital Melbourne (AU)
- Düsseldorf University Hospital (DE)
- Monash Alfred Psychiatry Research centre (AU)
- Azienda Socio Sanitaria Territoriale Grande Ospedale Metropolitano Niguarda (IT)
- Integrated Oncology (United States) (US)
- Institute of Oncology Research (CH)
- City of Hope (US)
- Dana-Farber Cancer Institute (US)
- IRCCS Ospedale San Raffaele (IT)
- Oxford University Hospitals NHS Trust (GB)
- Amsterdam University Medical Centers (NL)
- University Hospital Cologne (DE)
- Azienda Ospedaliera Nazionale SS. Antonio e Biagio e Cesare Arrigo (IT)
- Consorci Institut D'Investigacions Biomediques August Pi I Sunyer (ES)
- Weill Cornell Medicine (US)
- Heinrich Heine University Düsseldorf (DE)
- The Ohio State University (US)
- Monash University (AU)
- Università della Svizzera italiana (CH)
- University of Amsterdam (NL)
- Icahn School of Medicine at Mount Sinai (US)
Publication Details
- Journal
- Blood
- Published
- 2025-04-16
- DOI
- https://doi.org/10.1182/blood.2024028064
- Citations
- 9
- Primary Topic
- Chronic Lymphocytic Leukemia Research
- Type
- article
- Field-Weighted Citation Impact
- 14.63
Funders
- Conquer Cancer Foundation
- Amgen
- Eli Lilly and Company
- AstraZeneca
- Sanofi
- Gilead Sciences
- American Society of Clinical Oncology
- BeiGene
- Incyte
- Deutsche Forschungsgemeinschaft
- National Institutes of Health
- Genentech