Idiopathic multicentric Castleman disease in children: a single-center retrospective analysis
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...
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BMC
2025-01-01
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| Series: | BMC Pediatrics |
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| Online Access: | https://doi.org/10.1186/s12887-024-05347-0 |
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| author | Junye Du Jiafeng Yao Honghao Ma Li Li Ang Wei Liping Zhang Dong Wang Zhigang Li Rui Zhang Tianyou Wang |
| author_facet | Junye Du Jiafeng Yao Honghao Ma Li Li Ang Wei Liping Zhang Dong Wang Zhigang Li Rui Zhang Tianyou Wang |
| author_sort | Junye Du |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-fb939abb26ee40e1acf87b45ae2a2735 |
| institution | Kabale University |
| issn | 1471-2431 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Pediatrics |
| spelling | doaj-art-fb939abb26ee40e1acf87b45ae2a27352025-01-19T12:38:48ZengBMCBMC Pediatrics1471-24312025-01-012511910.1186/s12887-024-05347-0Idiopathic multicentric Castleman disease in children: a single-center retrospective analysisJunye Du0Jiafeng Yao1Honghao Ma2Li Li3Ang Wei4Liping Zhang5Dong Wang6Zhigang Li7Rui Zhang8Tianyou Wang9Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Disease in Children, Ministry of Education, Department of Hematology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthHematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Disease in Children, Ministry of Education, Department of Hematology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthHematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Disease in Children, Ministry of Education, Department of Hematology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthHematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Disease in Children, Ministry of Education, Department of Hematology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthHematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Disease in Children, Ministry of Education, Department of Hematology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthHematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Disease in Children, Ministry of Education, Department of Hematology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthHematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Disease in Children, Ministry of Education, Department of Hematology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthHematologic Disease Laboratory, Beijing Key Laboratory of Pediatric Hematology Oncology; National Key Discipline of Pediatrics (Capital Medical University); Key Laboratory of Major Disease in Children, Ministry of Education, Beijing Pediatric Research Institute; Hematology Center, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthHematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Disease in Children, Ministry of Education, Department of Hematology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthHematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Disease in Children, Ministry of Education, Department of Hematology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthAbstract 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.https://doi.org/10.1186/s12887-024-05347-0Castleman diseasePathogenesisTreatmentChildren |
| spellingShingle | Junye Du Jiafeng Yao Honghao Ma Li Li Ang Wei Liping Zhang Dong Wang Zhigang Li Rui Zhang Tianyou Wang Idiopathic multicentric Castleman disease in children: a single-center retrospective analysis BMC Pediatrics Castleman disease Pathogenesis Treatment Children |
| title | Idiopathic multicentric Castleman disease in children: a single-center retrospective analysis |
| title_full | Idiopathic multicentric Castleman disease in children: a single-center retrospective analysis |
| title_fullStr | Idiopathic multicentric Castleman disease in children: a single-center retrospective analysis |
| title_full_unstemmed | Idiopathic multicentric Castleman disease in children: a single-center retrospective analysis |
| title_short | Idiopathic multicentric Castleman disease in children: a single-center retrospective analysis |
| title_sort | idiopathic multicentric castleman disease in children a single center retrospective analysis |
| topic | Castleman disease Pathogenesis Treatment Children |
| url | https://doi.org/10.1186/s12887-024-05347-0 |
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