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<p>We investigated treatment outcomes, relapse risk, and survival in 104 consecutive patients with non-endemic Burkitt lymphoma (BL) diagnosed and treated in Kazakhstan between 2013 and 2024 in a real-world setting. This was a retrospective, clinically based study analyzing baseline characteristics, treatment regimens, response to first-line therapy, prognostic factors, relapse, and survival. The median age was 26 years (range 2–80), with a male predominance (64%). All cases underwent MYC rearrangement assessment, and EBV status was available in 80 patients, with 28% testing positive. Curative-intent therapy was delivered to 95 patients (91%), including R-BFM (44%), R-EPOCH (23%), R-CODOX-M/R-IVAC (18%), and R-Hyper-CVAD (15%); no patient underwent autologous stem cell transplantation. After a median follow-up of 57 months, the 3-year overall survival (OS) and progression-free survival (PFS) were 57% and 56%, respectively. Patients younger than 18 years, all treated with R-BFM, had significantly superior outcomes compared with adults, with 3-year OS and PFS of 82% and 83%, respectively (<i>p</i> &lt; 0.001). Among adults, patients treated with R-Hyper-CVAD or R-CODOX-M/R-IVAC had better OS than those treated with R-EPOCH (50% vs. 19%, <i>p</i> = 0.04). In univariate analysis, age ≥ 40 years, female sex, ECOG performance status ≥ 2, and platelet count &lt; 150&#xa0;g/dL were associated with inferior prognosis. In multivariate analysis, age ≥ 40 years remained the only independent adverse prognostic factor. The adapted Burkitt Lymphoma International Prognostic Index retained prognostic value. In conclusion, this large real-world study demonstrates excellent outcomes in pediatric patients treated with intensive immunochemotherapy but substantially inferior results in adults, particularly in refractory disease, highlighting a critical unmet clinical need in this population.</p>

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Treatment of Burkitt lymphoma in real-world setting: findings on 104 consecutive cases diagnosed and treated in Kazakhstan over the last decade

  • 期刊论文

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  • 发表日期：

    2026年3月19日

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  • Akmaral Jazyltayeva,
  • Nazarii Shokun,
  • Ayazhan Umutbayeva,
  • Luana Conte,
  • Raigul Ramazanova,
  • Vadim Kemaikin,
  • Azat Karabekov,
  • Aidana Shalabay,
  • Aigerim Koshkarbayeva,
  • Nurbergen Kemelbekov,
  • Madina Zhumabay,
  • Fariza Shokubayeva,
  • Yana Stepanihyna,
  • Massimo Federico,
  • Saule Gabbasova

摘要

We investigated treatment outcomes, relapse risk, and survival in 104 consecutive patients with non-endemic Burkitt lymphoma (BL) diagnosed and treated in Kazakhstan between 2013 and 2024 in a real-world setting. This was a retrospective, clinically based study analyzing baseline characteristics, treatment regimens, response to first-line therapy, prognostic factors, relapse, and survival. The median age was 26 years (range 2–80), with a male predominance (64%). All cases underwent MYC rearrangement assessment, and EBV status was available in 80 patients, with 28% testing positive. Curative-intent therapy was delivered to 95 patients (91%), including R-BFM (44%), R-EPOCH (23%), R-CODOX-M/R-IVAC (18%), and R-Hyper-CVAD (15%); no patient underwent autologous stem cell transplantation. After a median follow-up of 57 months, the 3-year overall survival (OS) and progression-free survival (PFS) were 57% and 56%, respectively. Patients younger than 18 years, all treated with R-BFM, had significantly superior outcomes compared with adults, with 3-year OS and PFS of 82% and 83%, respectively (p < 0.001). Among adults, patients treated with R-Hyper-CVAD or R-CODOX-M/R-IVAC had better OS than those treated with R-EPOCH (50% vs. 19%, p = 0.04). In univariate analysis, age ≥ 40 years, female sex, ECOG performance status ≥ 2, and platelet count < 150 g/dL were associated with inferior prognosis. In multivariate analysis, age ≥ 40 years remained the only independent adverse prognostic factor. The adapted Burkitt Lymphoma International Prognostic Index retained prognostic value. In conclusion, this large real-world study demonstrates excellent outcomes in pediatric patients treated with intensive immunochemotherapy but substantially inferior results in adults, particularly in refractory disease, highlighting a critical unmet clinical need in this population.