Department I of Internal Medicine and Center of Integrated Oncology Aachen, University of Cologne, Bonn, Cologne, Duesseldorf, Cologne, Germany.
Department of Hematology and Oncology, Evangelisches Krankenhaus Hamm, Hamm, Germany.
Leukemia. 2021 Jan;35(1):169-176. doi: 10.1038/s41375-020-0797-x. Epub 2020 Mar 17.
Richter transformation (RT) is defined as development of aggressive lymphoma in patients (pts) with CLL. The incidence rates of RT among pts with CLL range from 2 to 10%. The aim of this analysis is to report the frequency, characteristics and outcomes of pts with RT enrolled in trials of the GCLLSG. A total of 2975 pts with advanced CLL were reviewed for incidence of RT. Clinical, laboratory, and genetic data were pooled. Time-to-event data, starting from time of CLL diagnosis, of first-line therapy or of RT diagnosis, were analyzed by Kaplan-Meier methodology. One hundred and three pts developed RT (3%): 95 pts diffuse large B-cell lymphoma (92%) and eight pts Hodgkin lymphoma (8%). Median observation time was 53 months (interquartile range 38.1-69.5). Median OS from initial CLL diagnosis for pts without RT was 167 months vs 71 months for pts with RT (HR 2.64, CI 2.09-3.33). Median OS after diagnosis of RT was 9 months. Forty-seven pts (46%) received CHOP-like regimens for RT treatment. Three pts subsequently underwent allogeneic and two pts autologous stem cell transplantation. Our findings show that within a large cohort of GCLLSG trial participants, 3% of the pts developed RT after receiving first-line chemo- or chemoimmunotherapy. This dataset confirms the ongoing poor prognosis and high mortality associated with RT.
里希特转化（RT）定义为慢性淋巴细胞白血病（CLL）患者中侵袭性淋巴瘤的发展。CLL 患者中 RT 的发生率范围为 2%至 10%。本分析的目的是报告 GCLLSG 试验中纳入的 RT 患者的频率、特征和结局。对 2975 例晚期 CLL 患者进行了 RT 发生率的回顾性分析。汇总了临床、实验室和遗传学数据。从 CLL 诊断、一线治疗或 RT 诊断开始，采用 Kaplan-Meier 方法分析时间事件数据。103 例患者发生 RT（3%）：95 例弥漫性大 B 细胞淋巴瘤（92%）和 8 例霍奇金淋巴瘤（8%）。中位观察时间为 53 个月（四分位距 38.1-69.5）。无 RT 患者从初始 CLL 诊断到 OS 的中位时间为 167 个月，而 RT 患者为 71 个月（HR 2.64，95%CI 2.09-3.33）。RT 诊断后的中位 OS 为 9 个月。47 例（46%）患者接受 CHOP 样方案治疗 RT。3 例患者随后接受了异基因和 2 例自体干细胞移植。我们的研究结果表明，在 GCLLSG 试验参与者的大型队列中，3%的患者在接受一线化疗或化疗免疫治疗后发生 RT。该数据集证实了 RT 相关的持续不良预后和高死亡率。
