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儿童伯基特淋巴瘤的临床特征、结局和治疗挑战:单中心经验。

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

机构信息

Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

Department of Haematology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

出版信息

Pediatr Hematol Oncol. 2021 May;38(4):305-318. doi: 10.1080/08880018.2020.1865493. Epub 2021 Mar 1.

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

本研究旨在评估在中低收入国家(LMIC)中,根据强化化疗方案治疗儿科伯基特淋巴瘤病例的临床病理特征、治疗结果和面临的挑战。这是一项针对 2015 年 1 月至 2019 年 9 月期间采用 Lymphomes Malins B(LMB)96 化疗方案治疗的儿科伯基特淋巴瘤病例(年龄<18 岁)的单中心回顾性分析。共分析了 40 例患者,中位年龄为 11.5 岁(范围 4-18 岁),男女比例为 4.7:1。患者分为不同的 LMB 风险组:A-3(7.5%)、B-31(77.5%)和 C-06(15%)。25(62.5%)例患者在就诊时存在腹部疾病。不同治疗风险组的生存分析显示,A 组的总生存率(OS)为 100%,B 组为 87%±6.1%,C 组为 44.4%±16.2%,差异具有统计学意义( 值=0.016)。多因素分析影响生存的预后因素显示,CNS 受累( 值=0.03)和诊断至治疗开始的中位时间超过 30 天( 值=0.04)与不良预后显著相关。培养阳性发热性中性粒细胞减少症的发生率为 28.2%,其中 69.2%的感染是由耐碳青霉烯类的革兰氏阴性菌引起的。在我们的研究中,尽管 A 组和 B 组患者的结果与 LMB 96 治疗结果相当,但 C 组患者的结果较差,主要是由于就诊时疾病处于晚期且诊断延迟。我们在研究中面临的关键挑战是诊断延迟、治疗费用负担、营养状况不佳和高感染相关死亡率。

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