International consensus statement on diagnosis, evaluation, and research of Richter transformation: the ERIC recommendations
Abstract
Abstract 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.
Article Details
Authors (29)
Adam S. Kittai
1Division of Hematology and Medical Oncology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY
Monia Marchetti
15Azienda Ospedaliera Universitaria di Alessandria, Alessandria, Italy
Othman Al-Sawaf
Ohad Benjamini
Chaim Sheba Medical Center, Ramat Gan, Israel
Alexey V. Danilov
Matthew S. Davids
1Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA
Barbara Eichhorst
Department I of Internal Medicine, Center of Integrated Oncology Aachen Bonn Cologne Düsseldorf, University Hospital of Cologne, Cologne, Germany
Toby A. Eyre
8Department of Hematology, Oxford University Hospitals NHS Foundation Trust, Churchill Cancer Center, Oxford, United Kingdom
Anna Maria Frustaci
ASST Grande Ospedale Metropolitano Niguarda, Milan
Michael Hallek
Department I of Internal Medicine, Center of Integrated Oncology Aachen Bonn Cologne Düsseldorf, University Hospital of Cologne, Cologne, Germany
Paul J. Hampel
13Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN
Yair Herishanu
13Faculty of Medicine, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel
Rodney J. Hicks
Arnon P. Kater
Amsterdam University Medical Center location University of Amsterdam, Department of Hematology
Rebecca L. King
14Division of Hematopathology, Mayo Clinic, Rochester, MN
José I. Martin-Subero
Carolyn Owen
6University of Calgary, Calgary, Canada
Erin Parry
1Dana-Farber Cancer Institute, Medical Oncology, Boston, United States
Maurilio Ponzoni
Davide Rossi
Institute of Oncology Research, Bellinzona, Switzerland
Tanya Siddiqi
City of Hope Orange County, Irvine, California, United States
Stephan Stilgenbauer
Division of CLL, Department of Internal Medicine III, Ulm University, Ulm, Germany
Constantine S. Tam
40Department of Haematology, Alfred Hospital and Monash University, Melbourne, VIC, Australia
Elisa ten Hacken
Philip A. Thompson
24Peter MacCallum Cancer Centre and The Royal Melbourne Hospital, Melbourne, VIC, Australia
William Wierda
1The University of Texas MD Anderson Cancer Center, Houston, United States
Gianluca Gaidano
13Department of Translational Medicine, University of Eastern Piedmont, Novara, Italy
Jennifer A. Woyach
5Division of Hematology, The Ohio State University Comprehensive Cancer Center, Columbus, OH
Paolo Ghia
School of Medicine, Università Vita Salute San Raffaele, Milan