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Idiopathic multicentric Castleman disease in children: a single-center retrospective analysis
Junye Du1, Jiafeng Yao1, Honghao Ma1
1Hematology 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 Health, Nanlishi Road No. 56, Xicheng District, Beijing, 100045, China.
Insights
Pediatric idiopathic multicenter Castleman disease (iMCD) presents with systemic symptoms and enlarged lymph nodes. Treatments combining anti-IL-6 therapy, hormones, and lenalidomide show therapeutic effects in children with iMCD.
Area of Science:
- Pediatric Hematology
- Oncology
- Immunology
Background:
- Idiopathic multicenter Castleman disease (iMCD) is a rare lymphoproliferative disorder.
- Understanding its clinical spectrum in children is crucial for timely diagnosis and management.
Purpose of the Study:
- To investigate the clinical features, pathological subtypes, treatment strategies, and prognosis of pediatric iMCD.
- To evaluate the effectiveness of current therapeutic approaches.
Main Methods:
- Retrospective analysis of 9 pediatric iMCD cases diagnosed between January 2017 and September 2023.
- Data collected included demographics, clinical presentation, laboratory findings, pathological types, treatments, and outcomes.
Main Results:
- The median age of onset was 11 years (range 2-15).
- Pathological types included plasma cell (3), mixed (1), and hyaline vascular (5).
- Most patients received chemotherapy; 7/9 showed improvement, 1/9 stable, and 1/9 active disease after a median follow-up of 26 months.
Conclusions:
- Pediatric iMCD commonly manifests with systemic symptoms and lymphadenopathy.
- Anti-IL-6-based therapies, combined with agents like lenalidomide and corticosteroids, demonstrate efficacy in treating pediatric iMCD.
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.
