International consensus statement on diagnosis, evaluation, and research of Richter transformation: the ERIC recommendations

Kittai, AS; Eichhorst, B; Marchetti, M; Hallek, M; Al-Sawaf, O; Martin-Subero, JI; Benjamini, O; Parry, E; Danilov, AV; Ten Hacken, E; Davids, MS; Wierda, W; Eyre, TA; Frustaci, AM; Hampel, PJ; Herishanu, Y; Hicks, RJ; Kater, AP; King, RL; Owen, C; Ponzoni, M; Rossi, D; Siddiqi, T; Stilgenbauer, S; Tam, CS; Thompson, PA; Gaidano, G; Woyach, JA; Ghia, P

Author(s): Kittai, AS; Eichhorst, B; Marchetti, M; Hallek, M; Al-Sawaf, O; Martin-Subero, JI; Benjamini, O; Parry, E; Danilov, AV; Ten Hacken, E; Davids, MS; Wierda, W; Eyre, TA; Frustaci, AM; Hampel, PJ; Herishanu, Y; Hicks, RJ; Kater, AP; King, RL; Owen, C; Ponzoni, M; Rossi, D; Siddiqi, T; Stilgenbauer, S; Tam, CS; Thompson, PA; Gaidano, G; Woyach, JA; Ghia, P;
Details: Publication Year 2025-07-17,Volume 146,Issue #3,Page 291-303
Journal Title: Blood
Publication Type: Guideline
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.
Publisher: American Society of Hematology
Keywords: Humans; *Cell Transformation, Neoplastic/pathology; Consensus; *Leukemia, Lymphocytic, Chronic, B-Cell/diagnosis/therapy/pathology; *Lymphoma, Large B-Cell, Diffuse/diagnosis/therapy/pathology
Department(s): Haematology
Publisher's Version: https://doi.org/10.1182/blood.2024028064
Terms of Use/Rights Notice: Refer to copyright notice on published article.

Creation Date: 2025-05-13 07:45:02

Last Modified: 2025-08-22 08:45:17