Abstract

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 <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%; (p value = .016). On multivariate analysis of prognostic factors affecting survival, CNS involvement (p value = .03) and median time from diagnosis to treatment initiation more than 30 days (p 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.

摘要

进行这项研究的目的是评估在强化化疗方案治疗小儿Burkitt淋巴瘤病例期间，中低收入国家（LMIC）的临床病理特征，治疗结果和面临的挑战。这是对2015年1月至2019年9月间使用Lymphomes Malins B（LMB）96化疗方案统一处理的小儿Burkitt淋巴瘤病例（年龄<18岁）进行的单中心回顾性分析。分析了40例中位年龄为11.5岁的患者（范围4 –18岁），男女之比= 4.7：1。属于不同LMB风险组的患者为：A-3（7.5％），B-31（77.5％）和C-06（15％）。25例患者中有62.5％患有腹部疾病。不同治疗风险组的生存分析显示，A组100％，B组87％±6.1％和C-44.4％±16.2％之间的平均总生存率（OS）有统计学意义。（p值= 0.016）。对影响生存率，CNS受累（p值= .03）和从诊断到开始治疗的中位时间超过30天的多因素分析（p值= 0.04）与不良预后显着相关。培养阳性的发热性中性粒细胞减少症发作的发生率为28.2％，其中69.2％的感染是由于对碳青霉烯耐药的革兰氏阴性菌引起的。在我们的研究中，尽管风险组A和B的患者的结果与LMB 96的治疗结果相当，但风险组C的结果却相当差，这主要是由于就诊时疾病进展和诊断延迟。我们在队列研究中面临的关键挑战是诊断延迟，治疗费用负担得起，营养状况差以及与感染相关的死亡率高。