Division of Oncology and Hematology, University of Nebraska Medical Center, Omaha, NE, 68198, USA.
Department of Internal Medicine, Sanford School of Medicine, University of South Dakota, Vermillion, SD, USA.
Ann Hematol. 2021 Oct;100(10):2513-2519. doi: 10.1007/s00277-021-04603-y. Epub 2021 Jul 19.
Richter's transformation (RT) is a rare complication arising in patients with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) and is associated with an overall dismal outcome. The rarity of this entity poses many challenges in understanding its biology and outcomes seen and the optimal treatment approach. We utilized the SEER (Surveillance, Epidemiology and End Results) database to identify patients diagnosed with CLL/SLL between 2000 and 2016 and subsequently had a diagnosis of diffuse large B-cell lymphoma (DLBCL) or Hodgkin lymphoma (HL), thus capturing those who experienced an RT event. We compared the outcomes of those patients to those of patients in the database diagnosed with DLBCL without a preceding CLL/SLL diagnosis. We identified 530 patients who developed RT out of 74,116 patients diagnosed with CLL/SLL in the specified period. The median age at RT diagnosis was 66 years, and the median time from CLL/SLL diagnosis to RT development was roughly 4 years. Patients with RT had a dismal outcome with median overall survival of 10 months. We identified advanced Ann Arbor stage (III/IV) and prior treatment for CLL as predictors of worse outcome in patients with RT. Our study represents the largest dataset of patients with CLL/SLL and RT and adds to the existing literature indicating the poor outcomes for those patients.
里希特转化(RT)是慢性淋巴细胞白血病/小淋巴细胞淋巴瘤(CLL/SLL)患者中罕见的并发症,与总体预后不良相关。这种疾病的罕见性给理解其生物学和观察到的结果以及最佳治疗方法带来了许多挑战。我们利用 SEER(监测、流行病学和最终结果)数据库,确定了 2000 年至 2016 年间诊断为 CLL/SLL 的患者,随后诊断为弥漫性大 B 细胞淋巴瘤(DLBCL)或霍奇金淋巴瘤(HL),从而捕捉到经历 RT 事件的患者。我们将这些患者的结局与数据库中未诊断为 CLL/SLL 但诊断为 DLBCL 的患者的结局进行了比较。我们在指定期间内确定了 530 名从 74116 名 CLL/SLL 患者中发展为 RT 的患者。RT 诊断时的中位年龄为 66 岁,从 CLL/SLL 诊断到 RT 发展的中位时间约为 4 年。发生 RT 的患者预后极差,中位总生存期为 10 个月。我们发现晚期 Ann Arbor 分期(III/IV)和 CLL 的先前治疗是 RT 患者预后不良的预测因素。我们的研究代表了 CLL/SLL 和 RT 患者的最大数据集,并补充了表明这些患者预后不良的现有文献。