TY - JOUR
T1 - Burkitt Lymphoma International Prognostic Index
AU - Olszewski, Adam J
AU - Jakobsen, Lasse H
AU - Collins, Graham P
AU - Cwynarski, Kate
AU - Bachanova, Veronika
AU - Blum, Kristie A
AU - Boughan, Kirsten M
AU - Bower, Mark
AU - Dalla Pria, Alessia
AU - Danilov, Alexey
AU - David, Kevin A
AU - Diefenbach, Catherine
AU - Ellin, Fredrik
AU - Epperla, Narendranath
AU - Farooq, Umar
AU - Feldman, Tatyana A
AU - Gerrie, Alina S
AU - Jagadeesh, Deepa
AU - Kamdar, Manali
AU - Karmali, Reem
AU - Kassam, Shireen
AU - Kenkre, Vaishalee P
AU - Khan, Nadia
AU - Kim, Seo-Hyun
AU - Klein, Andreas K
AU - Lossos, Izidore S
AU - Lunning, Matthew A
AU - Martin, Peter
AU - Martinez-Calle, Nicolas
AU - Montoto, Silvia
AU - Naik, Seema
AU - Palmisiano, Neil
AU - Peace, David
AU - Phillips, Elizabeth H
AU - Phillips, Tycel J
AU - Portell, Craig A
AU - Reddy, Nishitha
AU - Santarsieri, Anna
AU - Sarraf Yazdy, Maryam
AU - Smeland, Knut B
AU - Smith, Scott E
AU - Smith, Stephen D
AU - Sundaram, Suchitra
AU - Zayac, Adam S
AU - Zhang, Xiao-Yin
AU - Zhu, Catherine
AU - Cheah, Chan Y
AU - El-Galaly, Tarec C
AU - Evens, Andrew M
AU - The Burkitt Lymphoma International Prognostic Index consortium
PY - 2021/4/1
Y1 - 2021/4/1
N2 - 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.
AB - 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.
U2 - 10.1200/JCO.20.03288
DO - 10.1200/JCO.20.03288
M3 - Journal article
C2 - 33502927
SN - 0732-183X
VL - 39
SP - 1129
EP - 1138
JO - Journal of Clinical Oncology
JF - Journal of Clinical Oncology
IS - 10
M1 - JCO2003288
ER -