Burkitt Lymphoma International Prognostic Index

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.

Burkitt Lymphoma Incidence in Five Continents

“…prognostic indices? [114][115][116] has been conducted in richer countries. The exclusion of poorer countries from BL research is ironic given that Denis Burkitt's epoch-making report was based on cases seen in Africa [12].…”

Genetic Subgroups Inform on Pathobiology in Adult and Pediatric Burkitt Lymphoma

“…The prognostication of survival outcomes in BL, especially with the respect to mutation status of a single predictor gene or gene panel, is under-studied compared to the association of BL survival with treatment 23 or clinical factors 24 . To determine the clinical relevance of newly discovered genetic subgroups in BL and their dominant features, we stratified patients using the most common mutations representative of the three BL-predominant subgroups, revealing significant associations between mutation status and survival outcomes.…”

Pre‐treatment total metabolic tumour volumes in lymphoma: Does quantity matter?

“…Total metabolic tumour volume was also investigated in Burkitt lymphoma (BL), in which most patients have advanced stage disease and/or bulky lesions at diagnosis. 80 A retrospective study of 65 BL patients found that using the SUV41% method, tMTV and TLG were independent prognostic factors for PFS and OS in multivariable analyses. 81 The optimal tMTV cut-off by ROC analysis was 231 ml.…”