🧬 Section 539.3 · Castleman Disease (Angiofollicular Lymph Node Hyperplasia)

Nelson Textbook of Pediatrics 22nd Edition | Castleman disease is a rare lymphoproliferative disorder. Unicentric Castleman disease (UCD): single enlarged lymph node, typically hyaline vascular type, cured by surgical excision. Multicentric Castleman disease (MCD): systemic symptoms (fever, weight loss, night sweats), cytopenias, organomegaly. Associated with HHV-8 (especially in HIV+), driven by IL-6. Treatment: rituximab for HHV-8+ MCD, siltuximab (anti-IL-6) for idiopathic MCD. TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis, organomegaly) is a subtype of MCD, treated with sirolimus.

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📋 30 Clinical Scenarios — Castleman Disease

📇 High‑Yield Review Cards (Castleman Disease)

🩺 Clinical Recognition: Castleman Disease

Select a presentation for diagnostic clues and management.

📋 Stepwise Management of Castleman Disease

🔑 Key Principles — Nelson Section 539.3
• Unicentric CD (UCD): Single lymph node region. Hyaline vascular histology. Asymptomatic or compressive. Treatment: surgical excision (curative).
• Multicentric CD (MCD): Systemic symptoms, cytopenias, organomegaly. Plasma cell histology. HHV-8+ MCD: rituximab ± ART. Idiopathic MCD (iMCD): siltuximab (anti-IL-6).
• TAFRO syndrome: Thrombocytopenia, anasarca, fever, reticulin fibrosis, organomegaly. Treat with sirolimus, rituximab, tocilizumab.
• Histology: Hyaline vascular (UCD), plasma cell (MCD). HHV-8 LANA1 staining positive in HHV-8+.

    ⚡ Reflex Prompts — Clinical Decisions in Castleman Disease

    📖 Summary: Castleman Disease — Nelson Section 539.3