Sýkorová Alice, Procházka Vít, Móciková Heidi, Janíková Andrea, Pytlík Robert, Belada David, Benešová Kateřina, Klener Pavel, Ďuraš Juraj, Smolej Lukáš, Campr Vít, Blahovcová Petra, Trněný Marek
4th Department of Internal Medicine-Hematology, University Hospital and Faculty of Medicine, Hradec Kralove, Czech Republic.
Department of Hemato-Oncology, Faculty of Medicine and Dentistry, Palacky University, Olomouc, Czech Republic.
Neoplasma. 2022 Dec;69(6):1466-1473. doi: 10.4149/neo_2022_221030N1064.
Burkitt lymphoma (BL) is a rare subtype of non-Hodgkin's lymphoma with an aggressive course. To refine the individual patient's prognosis, the International Prognostic Index for BL (BL-IPI) was recently developed and 4 risk factors (RF) were determined as optimal prognostic cut-off by multivariate analysis: age ≥40 years, lactate dehydrogenase >3× upper limit of normal, ECOG performance status ≥2, and central nervous system involvement. The BL-IPI distinguishes 3 prognostic groups, low (without RF), intermediate (1 RF), and high risk (2-4 RF), with significant differences in survival. The aim of the current project was to perform an external validation of the BL-IPI in 101 patients from the Registry of Czech Lymphoma Study Group diagnosed between 1999 and 2016 (median age, 45 years). The median follow-up was 50.4 months. The induction treatment included rituximab plus chemotherapy in 82% and chemotherapy alone in 18%. The overall response rate was 78% and the complete remission rate was 73%. According to BL-IPI, low/intermediate/high risk was present in 21/35/45% of patients, showing high similarity to the training BL-IPI US (United States) dataset (18/36/46%). There were significant differences in progression-free survival (PFS) and overall survival (OS) between patients with high vs. intermediate risk (PFS: hazard ratio 0.16, 95% confidence interval 0.08-0.31, p<0.0001; OS: hazard ratio 0.17, 95% confidence interval 0.09-0.35, p<0.0001) but not between patients with low vs. intermediate risk. The 3-year OS probability according to BL-IPI with low/intermediate/high risk was 96/76/59% in the BL-IPI training dataset vs. 95/85/45% in our external validation cohort; the 3-year PFS probability with low/intermediate/high risk was 92/72/53% in the BL-IPI training dataset vs. 95/85/42% in our cohort. In summary, our external validation of the BL-IPI confirmed a good separation of high-risk patients, who have a poor prognosis and for whom the new therapeutic approaches are needed; patients with low and intermediate risk had favorable clinical outcomes, and differences between these groups were not significant, likely due to a small number of patients.
伯基特淋巴瘤（BL）是一种罕见的非霍奇金淋巴瘤亚型，病程侵袭性强。为了优化个体患者的预后，近期制定了伯基特淋巴瘤国际预后指数（BL-IPI），并通过多变量分析确定了4个风险因素（RF）作为最佳预后临界值：年龄≥40岁、乳酸脱氢酶>正常上限的3倍、东部肿瘤协作组（ECOG）体能状态≥2以及中枢神经系统受累。BL-IPI区分出3个预后组，低风险（无RF）、中风险（1个RF）和高风险（2 - 4个RF），生存率存在显著差异。本项目的目的是对101例1999年至2016年间在捷克淋巴瘤研究组登记处确诊的患者（中位年龄45岁）进行BL-IPI的外部验证。中位随访时间为50.4个月。诱导治疗中82%的患者接受利妥昔单抗联合化疗，18%的患者仅接受化疗。总缓解率为78%，完全缓解率为73%。根据BL-IPI，低/中/高风险患者分别占21%/35%/45%，与BL-IPI美国训练数据集（18%/36%/46%）高度相似。高风险与中风险患者之间的无进展生存期（PFS）和总生存期（OS）存在显著差异（PFS：风险比0.16，95%置信区间0.08 - 0.31，p<0.0001；OS：风险比0.17，95%置信区间0.09 - 0.35，p<0.0001），但低风险与中风险患者之间无显著差异。在BL-IPI训练数据集中，低/中/高风险患者的3年总生存概率分别为96%/76%/59%，而在我们的外部验证队列中为95%/85%/45%；低/中/高风险患者的3年无进展生存概率在BL-IPI训练数据集中分别为92%/72%/53%，在我们的队列中为95%/85%/42%。总之，我们对BL-IPI的外部验证证实了高风险患者的良好区分，这些患者预后较差，需要新的治疗方法；低风险和中风险患者临床结局良好，且这些组之间的差异不显著，可能是由于患者数量较少。
