Clinical profile, outcome and challenges in the management of pediatric Burkitt lymphoma: a single center experience

This study was conducted with the aim to assess the clinico-pathological profile, treatment outcomes and the challenges faced in Low Middle Income Countries (LMIC) during management of pediatric Burkitt lymphoma cases on intensive chemotherapy protocol. This was a single center retrospective analysis of pediatric Burkitt lymphoma cases (age &lt;18 years) managed uniformly with Lymphomes Malins B (LMB) 96 chemotherapy protocol between January 2015 and September 2019. 40 cases were analyzed with a median age 11.5 years (range 4–18 years) and male: female ratio =4.7:1. Patients belonging to different LMB risk groups were: A-3 (7.5%), B-31 (77.5%), and C-06 (15%). 25 (62.5%) patients had abdominal disease at presentation. The survival analysis of different treatment risk groups showed statistically significant difference in mean Overall Survival (OS) between group A-100%, group B- 87%±6.1% and group C-44.4%±16.2%; (<i>p</i> value = .016). On multivariate analysis of prognostic factors affecting survival, CNS involvement (<i>p</i> value = .03) and median time from diagnosis to treatment initiation more than 30 days (<i>p</i> value = .04) were significantly associated with poor outcome. Incidence of culture positive febrile neutropenia episodes was 28.2% of which 69.2% infections were caused due to carbapenem resistant gram-negative organisms. In our study, although the outcomes in risk group A and B patients were comparable to LMB 96 treatment results, the outcome in risk group C was considerably poor primarily due to advanced disease at presentation and delayed diagnosis. The critical challenges that we faced in our cohort were delayed diagnosis, treatment cost affordability, poor nutritional status, and high infection related mortality.

本研究旨在评估中低收入国家（Low Middle Income Countries, LMIC）在采用强化化疗方案管理儿童伯基特淋巴瘤病例时的临床病理特征、治疗结局及面临的挑战。本研究为单中心回顾性分析，纳入2015年1月至2019年9月期间，采用统一的恶性淋巴瘤B细胞（Lymphomes Malins B, LMB）96化疗方案管理的18岁以下儿童伯基特淋巴瘤病例。共分析40例病例，中位年龄11.5岁（范围4～18岁），男女性别比为4.7:1。不同LMB风险组的患者分布为：A组3例（7.5%）、B组31例（77.5%）及C组6例（15%）。25例（62.5%）患者就诊时存在腹部病变。不同治疗风险组的生存分析显示，A组100%、B组（87%±6.1%）与C组（44.4%±16.2%）患者的平均总生存期（Overall Survival, OS）差异具有统计学意义（p值=0.016）。对影响生存的预后因素进行多因素分析后发现，中枢神经系统（Central Nervous System, CNS）受累（p值=0.03）以及从确诊到开始治疗的中位时间超过30天（p值=0.04）与不良结局显著相关。血培养阳性的发热性中性粒细胞减少症发生率为28.2%，其中69.2%的感染由碳青霉烯类耐药革兰阴性菌所致。本研究中，尽管A组与B组患者的治疗结局与LMB 96方案的既往治疗结果相当，但C组患者的结局显著较差，这主要归因于就诊时已处于疾病晚期以及诊断延迟。本队列中面临的关键挑战包括诊断延迟、治疗费用负担能力不足、营养状况不佳以及较高的感染相关死亡率。