Department of Pediatric Hematology and Oncology, Collegium Medicum, Nicolaus Copernicus University Toruń, Antoni Jurasz University Hospital No. 1, Bydgoszcz, Poland;
Student Scientific Society, Department of Pediatric Hematology and Oncology, Collegium Medicum, Nicolaus Copernicus University Toruń, Antoni Jurasz University Hospital No. 1, Bydgoszcz, Poland.
In Vivo. 2024 Nov-Dec;38(6):2812-2819. doi: 10.21873/invivo.13761.
BACKGROUND/AIM: Pediatric patients with primary refractory or relapsed B-cell non-Hodgkin lymphoma (B-NHL) have highly unfavorable prognosis. In this study, we retrospectively analyzed outcomes in pediatric B-NHL patients treated in a single center in Poland from 1995 to 2022, with emphasis on therapy results in patients with progression or relapse.
The primary objectives were a 5-year probability of overall survival (pOS) and a 5-year probability of event-free survival (pEFS). The secondary objectives involved the assessment of prognostic factors.
A total of 76 children were eligible for the analysis. The 5-year pOS was 76.7%, and the 5-year pEFS was 72.9%. At diagnosis, elevated lactate dehydrogenase activity, the presence of B symptoms, bone marrow, skeletal or mediastinal involvement, and stage IV disease were associated with inferior outcomes. Nine children experienced progression and four relapse. The 5-year pOS for patients with progression was 38.1%. Two patients treated with hematopoietic stem cell transplantation (HSCT) as part of salvage therapy survived. However, only one out of seven patients who were treated without HSCT survived. The 5-year pOS was 0.0% in patients with relapsed disease.
The most significant factor related to outcomes in pediatric B-NHL is therapy response, with a high mortality rate in children with refractory disease and relapse. There is no consensus on the salvage therapy approach; however, HSCT appears to be the optimal choice.
背景/目的：患有原发性难治或复发 B 细胞非霍奇金淋巴瘤（B-NHL）的儿科患者预后极差。本研究回顾性分析了波兰单一中心 1995 年至 2022 年治疗的儿科 B-NHL 患者的结果，重点关注进展或复发患者的治疗结果。
主要目标是 5 年总生存率（pOS）和 5 年无事件生存率（pEFS）的概率。次要目标涉及预后因素的评估。
共有 76 名儿童符合条件进行分析。5 年 pOS 为 76.7%，5 年 pEFS 为 72.9%。在诊断时，乳酸脱氢酶活性升高、B 症状存在、骨髓、骨骼或纵隔受累以及 IV 期疾病与预后不良相关。9 名儿童发生进展，4 名儿童复发。进展患者的 5 年 pOS 为 38.1%。两名接受造血干细胞移植（HSCT）作为挽救性治疗一部分的患者存活。然而，在未接受 HSCT 治疗的 7 名患者中只有 1 名存活。复发疾病患者的 5 年 pOS 为 0.0%。
与儿科 B-NHL 患者结局相关的最重要因素是治疗反应，难治性疾病和复发的儿童死亡率很高。对于挽救性治疗方法尚无共识；然而，HSCT 似乎是最佳选择。
