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

被引：0

作者：

Du, Junye ^{[1
]}

Yao, Jiafeng ^{[1
]}

Ma, Honghao ^{[1
]}

Li, Li ^{[1
]}

Wei, Ang ^{[1
]}

Zhang, Liping ^{[1
]}

Wang, Dong ^{[1
]}

Li, Zhigang ^{[2
,3
,4
]}

Zhang, Rui ^{[1
,5
]}

Wang, Tianyou ^{[1
,5
]}

机构：

[1] Capital Med Univ, Natl Key Discipline Pediat,Dept Hematol, Beijing Childrens Hosp,Key Lab Major Dis Children,, Hematol Ctr,Beijing Key Lab Pediat Hematol Oncol,N, Nanlishi Rd 56, Beijing 100045, Peoples R China

[2] Capital Med Univ, Natl Ctr Childrens Hlth, Hematol Dis Lab, Beijing Key Lab Pediat Hematol Oncol, Beijing 100045, Peoples R China

[3] Capital Med Univ, Natl Ctr Childrens Hlth, Natl Key Discipline Pediat, Beijing 100045, Peoples R China

[4] Capital Med Univ, Beijing Childrens Hosp, Hematol Ctr, Natl Ctr Childrens Hlth, Beijing 100045, Peoples R China

[5] Capital Med Univ, Beijing childrens Hosp, Dept Hematol, Nanlishi Rd 56, Beijing 100045, Peoples R China

来源：

BMC PEDIATRICS | 2025年 / 25卷 / 01期

关键词：

Castleman disease; Pathogenesis; Treatment; Children; TREATMENT GUIDELINES; INTERLEUKIN-6; TOCILIZUMAB;

D O I：

10.1186/s12887-024-05347-0

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

ObjectiveTo investigate the clinical features, pathological phenotype, treatment and prognosis of idiopathic multicenter Castleman disease (iMCD)in children.MethodsFrom 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.ResultsA 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.ConclusionChildren 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.

引用

页数：9

共 35 条

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