慢性淋巴细胞白血病患者的里希特转化：一项全国性的流行病学研究。
Richter's transformation in patients with chronic lymphocytic leukaemia: a Nationwide Epidemiological Study.
机构信息
Department of Haematology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.
Department of Haematology, Roskilde University, Roskilde, Denmark.
出版信息
Leuk Lymphoma. 2020 Jun;61(6):1435-1444. doi: 10.1080/10428194.2020.1719092. Epub 2020 Feb 7.
Richter's transformation (RT) refers to the development of an aggressive lymphoma in patients with chronic lymphocytic leukemia (CLL). Approximately, 2-10% of patients with CLL develop RT, most often as diffuse large B-cell lymphoma. To assess the incidence of RT, we examined risk factors for RT and death among patients with RT in a nationwide CLL cohort (from 2008 to 2016). Among 3772 patients, 113 had biopsy-proven RT. With a median follow-up of 4.3 years, the 5-year cumulative incidence of RT was 2.8%. Advanced Binet stage (B/C) (<.001), unmutated IGHV (<.001), and del(17p) (<.001) were independently associated with risk of developing RT. Half of the patients with RT (49%) were treatment-naïve prior to transformation and demonstrated longer survival after RT compared to patients previously treated for CLL (6.1 vs. 2.8 years, =.03). Whether this finding could be explained by a higher proportion of clonally unrelated RT among treatment-naïve patients, remain to be addressed.
里希特转化（RT）是指慢性淋巴细胞白血病（CLL）患者中侵袭性淋巴瘤的发展。大约有 2-10%的 CLL 患者发生 RT，最常见的是弥漫性大 B 细胞淋巴瘤。为了评估 RT 的发生率，我们在一项全国性的 CLL 队列中（2008 年至 2016 年），研究了发生 RT 的患者的 RT 和死亡的风险因素。在 3772 名患者中，有 113 名经活检证实发生 RT。中位随访 4.3 年后，RT 的 5 年累积发生率为 2.8%。晚期 Binet 分期（B/C）（<.001）、IGHV 未突变（<.001）和 del(17p)（<.001）与发生 RT 的风险独立相关。一半的 RT 患者（49%）在转化前未经治疗，与之前接受过 CLL 治疗的患者相比，RT 后生存时间更长（6.1 年 vs. 2.8 年，=.03）。未经治疗的患者中是否存在更多非克隆相关的 RT，从而导致这一发现，仍有待解决。
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