Idiopathic Multicentric Castleman Disease in Children

The aim of this study was to examine the clinical characteristics, pathological types, treatment approaches, and outcomes of idiopathic multicentric Castleman disease (iMCD) in children.

Data regarding demographics, laboratory results, treatment methods, and prognosis were collected for children diagnosed with iMCD from January 2017 to September 2023.

The outcome of the findings revealed:

• The study included 9 children, with a median age of onset at 11 years (ranging from 2 to 15 years), comprising 6 males and 3 females.
• Pathological classifications revealed 3 cases of plasma cell type, 1 case of mixed type, and 5 cases of hyaline vascular type.
• All 9 children underwent various chemotherapy regimens. 
• The median follow-up duration was 26 months (ranging from 13 to 58 months), with no fatalities reported. 
• Improvements were seen in 7 out of 9 cases, 1 case remained stable, and 1 case exhibited active disease.

To conclude, children with MCD commonly present with systemic symptoms, including lymph node enlargement and associated compression being the predominant features, followed by fever and malaise. Treatment with anti-IL-6 therapies, in combination with corticosteroids and lenalidomide, has demonstrated a specific therapeutic benefit for MCD.

Source: Du J, Yao J, Ma H. et al. Idiopathic multicentric Castleman disease in children: a single-center retrospective analysis. BMC Pediatr. 2025;25(38). https://doi.org/10.1186/s12887-024-05347-0