The Burkitt Lymphoma International Prognostic Index (BL-IPI) is an effective risk-stratification tool for patients with the sporadic and immunodeficiency-related adult subtypes of the disease.
A study, published in the Journal of Clinical Oncology, showed the BL-IPI is capable of identifying more low risk patients whose treatment might be de-escalated, an intermediate risk group, and high risk patients requiring more novel treatments.
The BL-IPI was developed in a US cohort of 641 patients and validated in a pooled international cohort of 457 patients from Australia, Canada, Denmark, Norway, Sweden and the UK.
The median age of the patients was 47 years, 77% were male and 23% were HIV-positive.
In the final model, age ≥40 years, ECOG ≥2, LDH >3 x ULN, and CNS involvement were the cut-off points prognostic for both PFS and OS.
The researchers noted that cutoffs useful for DLBCL and follicular lymphoma, such as age >60 years and LDH >ULN, were not optimal in Burkitt lymphoma (BL).
The study reported the BL risk groups as low-risk (no risk factors; 18% of patients; 3-year PFS = 92%), intermediate risk (one risk factor; 36% of patients; 3-year PFS = 72%), and high-risk (≥ 2 factors; 46% of patients; 3-year PFS =53%).
“Patients with all four high-risk factors may have particularly poor PFS (< 40% at 3 years), but this group is so small (< 5% in both data sets) that it was more practical to include it with other higher-risk groups,” the study said.
Sub-group analyses showed BL-IPI was prognostic regardless of other factors such as HIV status, disease stage, or first-line chemotherapy regimen.
“The BL-IPI largely recapitulated the historical designation of low-risk BL from clinical trials; however, it identified a larger group with excellent survival,” the study said.
“The low-risk BL-IPI group is large enough to consider de-escalated treatment strategies.”
“Conversely, patients with high-risk BL-IPI need better treatment, and trials focused on this group could show significant survival increments with fewer subjects.”