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“Burkitt lymphoma has a unique biology and clinical course, and this type of cancer has historically lacked a standardized prognostic model,” said Andrew M. Evens, DO, MSc, FACP, Associate Director for Clinical Services and Director of the Lymphoma Program at Rutgers Cancer Institute and Medical Director of the Oncology Service Line at RWJBarnabas Health. “As part of an international collaborative, we developed and validated a simple yet robust prognostic model that accounts for the unique characteristics of Burkitt lymphoma to aid in risk stratification, interpretation of clinical trials and targeted development of innovative treatment approaches,” added Dr. Evens, who is senior and corresponding author of the work.
Derived from 30 United States cancer centers and validated via multiple cancer centers in Europe, Canada and Australia, the BL-IPI is a simplified stratification of baseline clinical factors and comparison of risk distribution in geographically diverse cohorts.
“This novel, validated index can help clinicians more accurately identify a prognosis for their adult Burkitt lymphoma patients, as well as advance targeted clinical research studies,” said Adam J. Olszewski, MD of the Lifespan Cancer Institute, associate professor of medicine at The Warren Alpert Medical School of Brown University in Providence, RI, and lead author of the study. “It may engender dual goals of de-escalating treatment intensity for patients with low-risk disease and improving survival rates for the high-risk group, the latter which has suboptimal outcomes with standard therapy and should be considered for novel therapeutic strategies.”
“In addition, the availability of BL-IPI may also foster inquiry into molecular differences that lead to divergent clinical outcomes, which are not well explained by the gross indicators of disease burden at diagnosis,” adds Evens, who is also a Professor of Medicine at Rutgers Robert Wood Johnson Medical School.
About Burkitt Lymphoma
Burkitt lymphoma is a rare, mature high-grade B-cell lymphoma that globally constitutes 2 percent of all adult non-Hodgkin lymphomas, with an estimated incidence of 11,285 cases per year (according to The Lancet and BMJ Open).1,2 BL is curable for many patients with intensive multi-agent immunochemotherapy, and has been classified into variably defined low- and high-risk groups. However, there has not been uniform agreement on clinical prognostication or reproducible risk stratification, which poses challenges to individualized therapy, interpretation of clinical trials and design of future studies.
About the Research
The researchers’ objective was to develop and validate a simple prognostic index specific to adult BL that would account for its unique features and be applicable across various geographic settings where immunochemotherapy regimens are applied as standard treatment. The BL-IPI was derived from a real-world dataset 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 dataset of patients treated in Europe, Canada and Australia between 2004 and 2019.
In the cohort of 633 patients with BL derived from the U.S. dataset, also known as the derivation cohort, the following were selected as equally weighted risk factors with independent prognostic value: 1) age greater than or equal to 40; 2) performance status greater than or equal to two; 3) serum lactate dehydrogenase greater than 3 times the upper limit of normal; and 4) central nervous system involvement. The resulting BL-IPI identified patient groups with low risk (0 risk factors, 18 percent of patients), intermediate risk (one factor, 36 percent of patients) and high risk (two or more factors, 46 percent of patients). The three groups had corresponding progression free survival (PFS) estimates of 92 percent, 76 percent and 59 percent, respectively, and 3-year overall survival estimates of 96 percent, 76 percent and 59 percent, respectively. The index differentiated these outcomes regardless of the patients’ HIV status, stage of disease or first-line chemotherapy regimen. In the external cohort of 457 BL patients treated in Europe, Canada and Australia used to validate the index, the patient characteristics, relative size of the BL-IPI groupings and outcome differentiation were consistent: 3-year PFS estimates of 96 percent, 82 percent and 63 percent for low-, intermediate- and high-risk BL, respectively.
Limitations of the study include its retrospective design and a lack of formal central pathology review, although each case was diagnosed by expert academic hematopathologists and strict World Health Organization (WHO) criteria were applied. Additionally, a prognostic index will not necessarily predict patient benefit from any therapy, and the patient group risk estimates derived from the BL-IPI assume that all patients were treated with standard therapeutic approaches. This may not apply to patients older than 65 whose outcomes principally depend on whether they have comorbidities impacting their tolerance for intensive treatment. Further information on study limitations and additional findings can be found here: https://ascopubs.org/doi/full/10.1200/JCO.20.03288
This work was presented in part as an oral presentation at the 62nd ASH Annual Meeting and Exposition, December 5-8, 2020.
About Rutgers Cancer Institute of New Jersey
As New Jersey’s only National Cancer Institute-designated Comprehensive Cancer Center, Rutgers Cancer Institute, together with RWJBarnabas Health, offers the most advanced cancer treatment options, including bone marrow transplantation, proton therapy, CAR T-cell therapy and complex surgical procedures. Along with clinical trials and novel therapeutics such as precision medicine and immunotherapy – many of which are not widely available – patients have access to these cutting-edge therapies at Rutgers Cancer Institute of New Jersey in New Brunswick, Rutgers Cancer Institute of New Jersey at University Hospital in Newark, as well as through RWJBarnabas Health facilities. To make a tax-deductible gift to support the Cancer Institute of New Jersey, call 848-932-8013 or visit https://cinj.org/giving.
References
1 Molyneux EM, Rochford R, Griffin B, et al: Burkitt’s lymphoma. Lancet 379:1234-44, 2012
2 Khan G, Fitzmaurice C, Naghavi M, et al: Global and regional incidence, mortality and disability-adjusted life-years for Epstein-Barr virus-attributable malignancies, 1990-2017. BMJ Open 10:e037505, 2020
