Kittai Adam S, Marchetti Monia, Al-Sawaf Othman, Benjamini Ohad, Danilov Alexey V, Davids Matthew S, Eichhorst Barbara, Eyre Toby A, Frustaci Anna Maria, Hallek Michael, Hampel Paul J, Herishanu Yair, Hicks Rodney J, Kater Arnon P, King Rebecca L, Martin-Subero Jose I, Owen Carolyn, Parry Erin, Ponzoni Maurilio, Rossi Davide, Siddiqi Tanya, Stilgenbauer Stephan, Tam Constantine S, Hacken Elisa Ten, Thompson Philip A, Wierda William, Gaidano Gianluca, Woyach Jennifer A, Ghia Paolo
Division of Hematology and Medical Oncology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY.
Hematology and Transplant Center, Azienda Ospedaliera Universitaria SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.
Blood. 2025 Jul 17;146(3):291-303. doi: 10.1182/blood.2024028064.
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.
里氏转化（RT）被定义为慢性淋巴细胞白血病/小淋巴细胞淋巴瘤（CLL）患者中出现的侵袭性淋巴瘤。尽管CLL领域已开发出新型疗法，但RT的预后较差。鉴于RT诊断程序和治疗理念方面的最新进展，在欧洲CLL研究倡议的协调下，一个国际专家小组针对该疾病的临床程序和未来研究制定了共识性建议。RT患者通常表现为临床迅速恶化、B症状加重、乳酸脱氢酶升高和/或淋巴结迅速肿大。对于疑似RT的患者，检查应包括正电子发射断层扫描-计算机断层扫描。应从可触及的病灶处进行切除活检，最好是氟脱氧葡萄糖摄取率最高的病灶，并分析是否存在侵袭性淋巴瘤。应明确与原始CLL克隆的分子关系。由于不存在针对RT的有效标准治疗方法，患者应在临床试验中接受治疗。应在早期评估RT和CLL的反应，并且在试验设计中应优先考虑生存终点。我们希望这些建议有助于协调临床和转化研究，并改善RT患者的预后。
