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, Umar Farooq, Tatyana A. Feldman, Alina S. Gerrie, Deepa Jagadeesh, Manali Kamdar, Reem KarmaliShireen Kassam, Vaishalee P. Kenkre, Nadia Khan, Seo Hyun Kim, Andreas K. Klein, Izidore S. Lossos, Matthew A. Lunning, Peter Martin, Nicolas Martinez-Calle, Silvia Montoto, Seema Naik, Neil Palmisiano, David Peace, Elizabeth H. Phillips, Tycel J. Phillips, Craig A. Portell, Nishitha Reddy, Anna Santarsieri, Maryam Sarraf Yazdy, Knut B. Smeland, Scott E. Smith, Stephen D. Smith, Suchitra Sundaram, Adam S. Zayac, Xiao Yin Zhang, Catherine Zhu, Chan Y. Cheah, Tarec C. El-Galaly, Andrew M. Evens

Research output: Contribution to journalArticlepeer-review

16 Scopus citations


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 . 33 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 (US)
Pages (from-to)1129-1138
Number of pages10
JournalJournal of Clinical Oncology
Issue number10
StatePublished - Apr 1 2021

Bibliographical note

Funding Information:
The work of Tarec C. El-Galaly was supported by Danish Cancer Society.

Publisher Copyright:
© 2021 by American Society of Clinical Oncology


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