Idiopathic multicentric Castleman disease in children: a single-center retrospective analysis.

IF 2 3区医学 Q2 PEDIATRICS

BMC Pediatrics Pub Date : 2025-01-17 DOI:10.1186/s12887-024-05347-0

Junye Du, Jiafeng Yao, Honghao Ma, Li Li, Ang Wei, Liping Zhang, Dong Wang, Zhigang Li, Rui Zhang, Tianyou Wang

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引用次数: 0

Abstract

Objective: To investigate the clinical features, pathological phenotype, treatment and prognosis of idiopathic multicenter Castleman disease (iMCD)in children.

Methods: From January 2017 to September 2023, basic information, laboratory tests, treatment and prognosis of children diagnosed with iMCD who attended Beijing Children's Hospital of Capital Medical University were collected.

Results: A total of 9 children were enrolled, with a median age of onset of median 11 (2-15) years, 6 males and 3 female. 3 cases were pathologically typed as plasma cell type, 1 case was mixed type, and the remaining 5 cases were hyaline vascular type. 9 children received different regimens of chemotherapy. The median follow-up time was 26 (13, 58) months, with no deaths, 7/9 cases showing improvement, 1/9 cases showing stable condition, and 1/9 cases showing active condition.

Conclusion: Children with multicentric CD often have systemic symptoms, lymph node enlargement and related compression symptoms are the most common manifestations, followed by fever, malaise and other systemic symptoms. Anti-IL-6-based therapy combined with hormones lenalidomide and other medications have a specific therapeutic effect on multicentric CD.

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儿童特发性多中心Castleman病：单中心回顾性分析

目的：探讨儿童特发性多中心Castleman病（iMCD）的临床特点、病理表型、治疗及预后。方法：收集2017年1月至2023年9月在首都医科大学附属北京儿童医院就诊的诊断为iMCD患儿的基本信息、实验室检查、治疗及预后。结果：共有9名儿童入组，中位发病年龄为11（2-15）岁，其中6名男性，3名女性。病理分型为浆细胞型3例，混合型1例，透明血管型5例。9名儿童接受了不同的化疗方案。中位随访时间26（13.58）个月，无死亡病例，7/9好转，1/9病情稳定，1/9病情活跃。结论：多中心性CD患儿多有全身性症状，淋巴结肿大及相关压迫症状是最常见的表现，其次为发热、不适等全身性症状。以抗il -6为基础的治疗联合激素来那度胺等药物对多中心性CD有特异性治疗效果。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

BMC Pediatrics PEDIATRICS-

CiteScore

3.70

自引率

4.20%

发文量

683

审稿时长

3-8 weeks

期刊介绍： BMC Pediatrics is an open access journal publishing peer-reviewed research articles in all aspects of health care in neonates, children and adolescents, as well as related molecular genetics, pathophysiology, and epidemiology.