Dataset Information

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.

SUBMITTER: Olszewski AJ

PROVIDER: S-EPMC9851706 | biostudies-literature | 2021 Apr

REPOSITORIES: biostudies-literature

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Publications

Burkitt Lymphoma International Prognostic Index.

Olszewski Adam J AJ Jakobsen Lasse H LH Collins Graham P GP Cwynarski Kate K Bachanova Veronika V Blum Kristie A KA Boughan Kirsten M KM Bower Mark M Dalla Pria Alessia A Danilov Alexey A David Kevin A KA Diefenbach Catherine C Ellin Fredrik F Epperla Narendranath N Farooq Umar U Feldman Tatyana A TA Gerrie Alina S AS Jagadeesh Deepa D Kamdar Manali M Karmali Reem R Kassam Shireen S Kenkre Vaishalee P VP Khan Nadia N Kim Seo-Hyun SH Klein Andreas K AK Lossos Izidore S IS Lunning Matthew A MA Martin Peter P Martinez-Calle Nicolas N Montoto Silvia S Naik Seema S Palmisiano Neil N Peace David D Phillips Elizabeth H EH Phillips Tycel J TJ Portell Craig A CA Reddy Nishitha N Santarsieri Anna A Sarraf Yazdy Maryam M Smeland Knut B KB Smith Scott E SE Smith Stephen D SD Sundaram Suchitra S Zayac Adam S AS Zhang Xiao-Yin XY Zhu Catherine C Cheah Chan Y CY El-Galaly Tarec C TC Evens Andrew M AM

Journal of clinical oncology : official journal of the American Society of Clinical Oncology 20210127 10

<h4>Purpose</h4>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.<h4>Methods</h4>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 a ...[more]

PMID: 33502927

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Project description:BackgroundThe PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) study is a prospective analysis of an international database. Here we examine front-line treatments and quality of life (QoL) in patients with newly diagnosed mycosis fungoides (MF).ObjectivesTo identify (i) differences in first-line approaches according to tumour-nodes-metastasis-blood (TNMB) staging; (ii) parameters related to a first-line systemic approach and (iii) response rates and QoL measures.MethodsIn total, 395 newly diagnosed patients with early-stage MF (stage IA-IIA) were recruited from 41 centres in 17 countries between 1 January 2015 and 31 December 2018 following central clinicopathological review.ResultsThe most common first-line therapy was skin-directed therapy (SDT) (322 cases, 81·5%), while a smaller percentage (44 cases, 11·1%) received systemic therapy. Expectant observation was used in 7·3%. In univariate analysis, the use of systemic therapy was significantly associated with higher clinical stage (IA, 6%; IB, 14%; IIA, 20%; IA-IB vs. IIA, P < 0·001), presence of plaques (T1a/T2a, 5%; T1b/T2b, 17%; P < 0·001), higher modified Severity Weighted Assessment Tool (> 10, 15%; ≤ 10, 7%; P = 0·01) and folliculotropic MF (FMF) (24% vs. 12%, P = 0·001). Multivariate analysis demonstrated significant associations with the presence of plaques (T1b/T2b vs. T1a/T2a, odds ratio 3·07) and FMF (odds ratio 2·83). The overall response rate (ORR) to first-line SDT was 73%, while the ORR to first-line systemic treatments was lower (57%) (P = 0·027). Health-related QoL improved significantly both in patients with responsive disease and in those with stable disease.ConclusionsDisease characteristics such as presence of plaques and FMF influence physician treatment choices, and SDT was superior to systemic therapy even in patients with such disease characteristics. Consequently, future treatment guidelines for early-stage MF need to address these issues.

Dataset Information

Burkitt Lymphoma International Prognostic Index.

Purpose

Methods

Results

Conclusion

Publications

Burkitt Lymphoma International Prognostic Index.

Similar Datasets

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