Pediatric B-cell Non-Hodgkin Lymphoma: The Impact of Therapy Response and Relapse on Outcome. A Single-center Analysis.

IF 1.8 4区 医学 Q3 MEDICINE, RESEARCH & EXPERIMENTAL In vivo Pub Date : 2024-11-01 DOI:10.21873/invivo.13761
Joanna Stankiewicz, Anna Jabłońska, Paweł Treichel, Ewa Demidowicz, Jan Styczyński
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Abstract

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.

Patients and methods: 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.

Results: 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.

Conclusion: 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.

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小儿 B 细胞非霍奇金淋巴瘤:治疗反应和复发对预后的影响。单中心分析。
背景/目的:患有原发性难治性或复发性B细胞非霍奇金淋巴瘤(B-NHL)的小儿患者预后极差。在这项研究中,我们回顾性分析了1995年至2022年在波兰一个中心接受治疗的小儿B-NHL患者的预后,重点分析了病情进展或复发患者的治疗结果:主要目标是5年总生存概率(pOS)和5年无事件生存概率(pEFS)。次要目标包括评估预后因素:共有 76 名儿童符合分析条件。5年无事件生存概率为76.7%,5年无事件生存概率为72.9%。确诊时,乳酸脱氢酶活性升高,出现B症状,骨髓、骨骼或纵隔受累,以及疾病处于IV期,都与不良预后有关。九名患儿病情恶化,四名复发。病情恶化患者的 5 年生存率为 38.1%。作为挽救疗法的一部分,两名接受造血干细胞移植(HSCT)治疗的患者存活了下来。然而,在未接受造血干细胞移植治疗的七名患者中,只有一人存活。复发患者的5年生存率为0.0%:结论:与小儿B-NHL预后相关的最重要因素是治疗反应,难治性疾病和复发患儿的死亡率很高。对于挽救治疗方法,目前还没有达成共识;不过,造血干细胞移植似乎是最佳选择。
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来源期刊
In vivo
In vivo 医学-医学:研究与实验
CiteScore
4.20
自引率
4.30%
发文量
330
审稿时长
3-8 weeks
期刊介绍: IN VIVO is an international peer-reviewed journal designed to bring together original high quality works and reviews on experimental and clinical biomedical research within the frames of physiology, pathology and disease management. The topics of IN VIVO include: 1. Experimental development and application of new diagnostic and therapeutic procedures; 2. Pharmacological and toxicological evaluation of new drugs, drug combinations and drug delivery systems; 3. Clinical trials; 4. Development and characterization of models of biomedical research; 5. Cancer diagnosis and treatment; 6. Immunotherapy and vaccines; 7. Radiotherapy, Imaging; 8. Tissue engineering, Regenerative medicine; 9. Carcinogenesis.
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