Lifespan Cancer Institute, The Warren Alpert Medical School of Brown University, Providence, RI.
Department of Haematology, Aalborg University Hospital, Aalborg, Denmark.
J Clin Oncol. 2021 Apr 1;39(10):1129-1138. doi: 10.1200/JCO.20.03288. Epub 2021 Jan 27.
Burkitt lymphoma (BL) has unique biology and clinical course but lacks a standardized prognostic model. We developed and validated a novel prognostic index specific for BL to aid risk stratification, interpretation of clinical trials, and targeted development of novel treatment approaches.
We derived the BL International Prognostic Index (BL-IPI) from a real-world data set of adult patients with BL treated with immunochemotherapy in the United States between 2009 and 2018, identifying candidate variables that showed the strongest prognostic association with progression-free survival (PFS). The index was validated in an external data set of patients treated in Europe, Canada, and Australia between 2004 and 2019.
In the derivation cohort of 633 patients with BL, age ≥ 40 years, performance status ≥ 2, serum lactate dehydrogenase > 3× upper limit of normal, and CNS involvement were selected as equally weighted factors with an independent prognostic value. The resulting BL-IPI identified groups with low (zero risk factors, 18% of patients), intermediate (one factor, 36% of patients), and high risk (≥ 2 factors, 46% of patients) with 3-year PFS estimates of 92%, 72%, and 53%, respectively, and 3-year overall survival estimates of 96%, 76%, and 59%, respectively. The index discriminated outcomes regardless of HIV status, stage, or first-line chemotherapy regimen. Patient characteristics, relative size of the BL-IPI groupings, and outcome discrimination were consistent in the validation cohort of 457 patients, with 3-year PFS estimates of 96%, 82%, and 63% for low-, intermediate-, and high-risk BL-IPI, respectively.
The BL-IPI provides robust discrimination of survival in adult BL, suitable for use as prognostication and stratification in trials. The high-risk group has suboptimal outcomes with standard therapy and should be considered for innovative treatment approaches.
伯基特淋巴瘤（BL）具有独特的生物学和临床过程，但缺乏标准化的预后模型。我们开发并验证了一种新的 BL 特异性预后指数，以帮助风险分层、解释临床试验结果，并为新的治疗方法提供靶向开发。
我们从 2009 年至 2018 年期间在美国接受免疫化疗治疗的成人 BL 患者的真实世界数据集中得出 BL 国际预后指数（BL-IPI），确定了与无进展生存期（PFS）具有最强预后关联的候选变量。该指数在 2004 年至 2019 年期间在欧洲、加拿大和澳大利亚接受治疗的患者的外部数据集中进行了验证。
在 633 例 BL 患者的推导队列中，年龄≥40 岁、表现状态≥2、血清乳酸脱氢酶>3×正常值上限和 CNS 受累被选为具有独立预后价值的同等加权因素。由此产生的 BL-IPI 确定了低危（无风险因素，占患者的 18%）、中危（一个因素，占患者的 36%）和高危（≥2 个因素，占患者的 46%）三组，3 年 PFS 估计值分别为 92%、72%和 53%，3 年总生存率估计值分别为 96%、76%和 59%。该指数可区分无论 HIV 状态、分期或一线化疗方案如何的结局。在 457 例患者的验证队列中，患者特征、BL-IPI 分组的相对大小和结果区分度均一致，低危、中危和高危 BL-IPI 的 3 年 PFS 估计值分别为 96%、82%和 63%。
BL-IPI 对成人 BL 的生存提供了强大的区分能力，适用于临床试验中的预后和分层。高危组采用标准治疗方案效果不佳，应考虑采用创新治疗方法。
