Pharmaceutical Sciences Postgraduate Program, Federal University of Paraná, Curitiba, PR, Brazil.
Hematology Service, University Hospital Professor Polydoro Ernani de São Thiago, Federal University of Santa Catarina, Florianópolis, SC, Brazil.
Clin Lymphoma Myeloma Leuk. 2021 Aug;21(8):514-525. doi: 10.1016/j.clml.2021.03.012. Epub 2021 Apr 3.
Burkitt lymphoma (BL) is an aggressive hematologic cancer. This study synthetized the evidence about the efficacy and safety of chemotherapy treatments used in patients with BL using the World Health Organization classification.
A systematic review of interventional studies was performed. A search was carried out in PubMed, Scopus, and Web of Science, with additional manual and gray literature searches. The methodological quality of articles was assessed with the Newcastle-Ottawa scale.
We identified 1358 studies; 9 nonrandomized studies satisfied the eligibility criteria (n = 544 patients). The BL epidemiologic variants were sporadic BL (44.5%), endemic BL (47.2%), and immunodeficiency-associated BL (8.3%). Regarding chemotherapy protocols, 4 groups were identified: based on CODOX-M/IVAC (n = 4), EPOCH (n = 1), BFM (n = 1), and simplified treatment schemes used in African countries (n = 3). Most studies had moderate quality. Empirically and qualitatively, the best options for adults with sporadic BL were 'DA-EPOCH-R' (7-year overall survival [OS], 100%; 95% confidence interval [CI], 82-100), 'HDR + LD into CODOX-M/IVAC' (2-year OS, 84%), and 'RD-CODOX-M/IVAC' (4-year progression-free survival, 92%; 95% CI, 77-100); in pediatric patients, the 'BFM-NHL-90-like' showed promising results (3-year OS, 90%). For immunodeficiency-associated BL, the 'SC-EPOCH-RR' demonstrated a good therapeutic profile (6-year OS, 90%; 95% CI, 60-98). The 'Malawi 2012-2014' (1-year OS, 73%; 95% CI, 61-85) could be the treatment choice in endemic BL (African countries). The main adverse events were hematologic.
Selecting chemotherapy protocols for BL should be grounded in its epidemiologic variants. Further studies with greater methodological quality are needed to strengthen the evidence.
伯基特淋巴瘤（BL）是一种侵袭性血液系统恶性肿瘤。本研究综合了使用世界卫生组织分类的 BL 患者化疗治疗的疗效和安全性证据。
进行了干预性研究的系统评价。在 PubMed、Scopus 和 Web of Science 进行了检索，并进行了额外的手动和灰色文献检索。使用纽卡斯尔-渥太华量表评估文章的方法学质量。
我们确定了 1358 项研究；9 项非随机研究符合入选标准（n=544 例患者）。BL 的流行病学变异包括散发性 BL（44.5%）、地方性 BL（47.2%）和免疫缺陷相关 BL（8.3%）。关于化疗方案，我们确定了 4 组：基于 CODOX-M/IVAC（n=4）、EPOCH（n=1）、BFM（n=1）和简化的非洲国家使用的治疗方案（n=3）。大多数研究的质量为中等。经验性和定性地，散发性 BL 成人患者的最佳选择是“DA-EPOCH-R”（7 年总生存率[OS]，100%；95%置信区间[CI]，82-100）、“HDR+LD 入 CODOX-M/IVAC”（2 年 OS，84%）和“RD-CODOX-M/IVAC”（4 年无进展生存率，92%；95%CI，77-100）；儿科患者中，“BFM-NHL-90 样”显示出有前景的结果（3 年 OS，90%）。对于免疫缺陷相关 BL，“SC-EPOCH-RR”显示出良好的治疗效果（6 年 OS，90%；95%CI，60-98）。“马拉维 2012-2014 年”（1 年 OS，73%；95%CI，61-85）可能是地方性 BL（非洲国家）的治疗选择。主要不良事件为血液学毒性。
BL 化疗方案的选择应基于其流行病学变异。需要进一步开展方法学质量更高的研究来加强证据。
