Burkitt lymphoma: current understanding, unmet needs, and research directions.

IF 7.7 1区医学 Q1 HEMATOLOGY

Blood advances Pub Date : 2026-10-13 DOI:10.1182/bloodadvances.2024015620

Adiba Khan, Cristina López, Suzanne D Turner, Sam M Mbulaiteye

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引用次数: 0

Abstract

Abstract: Burkitt lymphoma (BL) is an aggressive B-cell lymphoma historically classified into endemic, sporadic, or immunodeficiency-associated BL based on clinical and epidemiological features. Recent genomic and epigenomic studies have refined this framework, distinguishing Epstein-Barr virus (EBV)-positive and EBV-negative BL molecular subtypes that cut across traditional clinical and epidemiological categories and exhibit distinct biological profiles. In high-income countries (HICs), BL is highly curable with intensive multiagent chemotherapy and antibody-based therapy. In contrast, survival in sub-Saharan Africa rarely exceeds 50%, reflecting diagnostic delays, limited access to effective treatment, and challenges in supportive care. This review summarizes the advances in BL epidemiology, molecular biology, diagnosis, and treatment, highlighting how genomic insights have identified novel biomarkers for earlier detection and monitoring, as well as potential targeted therapies with reduced toxicity. These advances present opportunities to develop scalable diagnostic tools and tailored therapeutic strategies to narrow survival disparities, particularly in resource-limited settings, where most patients occur, and among adults and patients with relapsed or refractory BL in HICs. We also outline key unanswered questions in BL epidemiology and pathogenesis, discuss opportunities for translational research, and emphasize the need for improved model systems to study therapeutic resistance to close the survival gap.

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伯基特淋巴瘤：目前的认识，未满足的需求和研究方向。

伯基特淋巴瘤（BL）是一种侵袭性b细胞淋巴瘤，历史上根据临床和流行病学特征分为地方性、散发性或免疫缺陷相关的BL。最近的基因组学和表观基因组学研究完善了这一框架，区分了ebv阳性和ebv阴性的BL分子亚型，这些亚型跨越了传统的临床和流行病学类别，并表现出不同的生物学特征。在高收入国家，通过强化多药化疗和基于抗体的治疗，BL是高度可治愈的。相比之下，撒哈拉以南非洲地区的生存率很少超过50%，这反映出诊断延误、获得有效治疗的机会有限以及在支持性护理方面存在挑战。本文综述了BL流行病学、分子生物学、诊断和治疗方面的进展，重点介绍了基因组学如何发现新的生物标志物，用于早期检测和监测，以及降低毒性的潜在靶向治疗。这些进展为开发可扩展的诊断工具和量身定制的治疗策略提供了机会，以缩小生存差距，特别是在资源有限的环境中，在大多数病例发生的地方，以及在高收入国家的成人和复发或难治性BL患者中。我们还概述了BL流行病学和发病机制中未解决的关键问题，讨论了转化研究的机会，并强调需要改进模型系统来研究治疗耐药性，以缩小生存差距。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Blood advances Medicine-Hematology

CiteScore

12.70

自引率

2.70%

发文量

840

期刊介绍： Blood Advances, a semimonthly medical journal published by the American Society of Hematology, marks the first addition to the Blood family in 70 years. This peer-reviewed, online-only, open-access journal was launched under the leadership of founding editor-in-chief Robert Negrin, MD, from Stanford University Medical Center in Stanford, CA, with its inaugural issue released on November 29, 2016. Blood Advances serves as an international platform for original articles detailing basic laboratory, translational, and clinical investigations in hematology. The journal comprehensively covers all aspects of hematology, including disorders of leukocytes (both benign and malignant), erythrocytes, platelets, hemostatic mechanisms, vascular biology, immunology, and hematologic oncology. Each article undergoes a rigorous peer-review process, with selection based on the originality of the findings, the high quality of the work presented, and the clarity of the presentation.