OBSOLETE: Pediatric Castleman disease

Pediatric Castleman disease (also known as Castleman disease occurring in children and adolescents) refers to a group of rare lymphoproliferative disorders characterized by abnormal overgrowth of lymph node tissue. This Orphanet entry (ORPHA:93682) is classified as obsolete, meaning it has been retired and its content has been reorganized under other current Orphanet classifications for Castleman disease. Castleman disease in general encompasses unicentric Castleman disease (UCD), which involves a single enlarged lymph node region, and multicentric Castleman disease (MCD), which affects multiple lymph node stations and is a systemic illness. In children, the unicentric form is more common and typically presents as an isolated enlarged lymph node mass, often in the chest, abdomen, or neck, that may cause compressive symptoms. Multicentric forms can present with systemic symptoms including fever, night sweats, weight loss, fatigue, enlarged liver and spleen, fluid accumulation (ascites, pleural effusions), and laboratory abnormalities such as anemia, elevated inflammatory markers, and hypergammaglobulinemia. The disease primarily affects the lymphatic and immune systems but can secondarily impact the hematopoietic system, liver, spleen, and kidneys. Treatment for unicentric disease is typically surgical excision, which is often curative. Multicentric Castleman disease may require systemic therapies including anti-interleukin-6 agents (such as siltuximab or tocilizumab), corticosteroids, rituximab, or combination chemotherapy. Patients with pediatric Castleman disease are now classified under the broader Castleman disease entries in current nosologies, and clinicians should refer to updated Orphanet entries for unicentric Castleman disease (ORPHA:160) or multicentric Castleman disease (ORPHA:93681) for the most current information.

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