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Olszewski AJ , Jakobsen LH , Collins GP , Cwynarski K , Bachanova V , Blum KA , Boughan KM , Bower M , Dalla Pria A , Danilov A , David KA , Diefenbach C , Ellin F , Epperla N , Farooq U , Feldman TA , Gerrie AS , Jagadeesh D , Kamdar M , Karmali R , Kassam S , Kenkre VP , Khan N , Kim SH , Klein AK , Lossos IS , Lunning MA , Martin P , Martinez-Calle N , Montoto S , Naik S , Palmisiano N , Peace D , Phillips EH , Phillips TJ , Portell CA , Reddy N , Santarsieri A , Sarraf Yazdy M , Smeland KB , Smith SE , Smith SD , Sundaram S , Zayac AS , Zhang XY , Zhu C , Cheah CY , El-Galaly TC , Evens AM
Burkitt Lymphoma International Prognostic Index
J Clin Oncol. 2021 Apr 1;39(10) :1129-1138
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PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.
1527-7755 Olszewski, Adam J Jakobsen, Lasse H Collins, Graham P Cwynarski, Kate Bachanova, Veronika Blum, Kristie A Boughan, Kirsten M Bower, Mark Dalla Pria, Alessia Danilov, Alexey David, Kevin A Diefenbach, Catherine Ellin, Fredrik Epperla, Narendranath Farooq, Umar Feldman, Tatyana A Gerrie, Alina S Jagadeesh, Deepa Kamdar, Manali Karmali, Reem Kassam, Shireen Kenkre, Vaishalee P Khan, Nadia Kim, Seo-Hyun Klein, Andreas K Lossos, Izidore S Lunning, Matthew A Martin, Peter Martinez-Calle, Nicolas Montoto, Silvia Naik, Seema Palmisiano, Neil Peace, David Phillips, Elizabeth H Phillips, Tycel J Portell, Craig A Reddy, Nishitha Santarsieri, Anna Sarraf Yazdy, Maryam Smeland, Knut B Smith, Scott E Smith, Stephen D Sundaram, Suchitra Zayac, Adam S Zhang, Xiao-Yin Zhu, Catherine Cheah, Chan Y El-Galaly, Tarec C Evens, Andrew M Journal Article United States J Clin Oncol. 2021 Jan 27:JCO2003288. doi: 10.1200/JCO.20.03288.