Burkitt Lymphoma International Prognostic Index

Adam J Olszewski, Lasse H Jakobsen, Graham P Collins, Kate Cwynarski, Veronika Bachanova, Kristie A Blum, Kirsten M Boughan, Mark Bower, Alessia Dalla Pria, Alexey Danilov, Kevin A David, Catherine Diefenbach, Fredrik Ellin, Narendranath Epperla, Tatyana A Feldman, Alina S Gerrie, Deepa Jagadeesh, Manali Kamdar, Reem Karmali, Shireen KassamVaishalee P Kenkre, Nadia Khan, Seo-Hyun Kim, Andreas K Klein, Izidore S Lossos, Matthew A Lunning, Nicolas Martinez-Calle, Silvia Montoto, Seema Naik, Neil Palmisiano, David Peace, Tycel J Phillips, Craig A Portell, Nishitha Reddy, Anna Santarsieri, Maryam Sarraf Yazdy, Knut B Smeland, Suchitra Sundaram, Adam S Zayac, Catherine Zhu, Chan Y Cheah, Tarec C El-Galaly, Andrew M Evens

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Number of pages14
JournalJournal of clinical oncology : official journal of the American Society of Clinical Oncology
DOIs
Publication statusE-pub ahead of print - 27 Jan 2021

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