🧬 Section 539.2 · Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy)

Nelson Textbook of Pediatrics 22nd Edition | Rosai-Dorfman disease (RDD) is a rare, benign non-Langerhans cell histiocytosis. Classic presentation: massive bilateral, painless cervical lymphadenopathy. Extranodal involvement occurs in 43% (skin, nasal cavity, orbit, bone, CNS). Histology: S100+, CD1a-, emperipolesis (lymphocytes engulfed by histiocytes). Treatment: observation (spontaneous remission 20-50%), surgery for symptomatic lesions, sirolimus, corticosteroids, chemotherapy, MEK inhibitors for refractory disease.

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📋 30 Clinical Scenarios — Rosai-Dorfman Disease

📇 High‑Yield Review Cards (Rosai-Dorfman Disease)

🩺 Clinical Recognition: Rosai-Dorfman Disease

Select a presentation for diagnostic clues and management.

📋 Stepwise Management of Rosai-Dorfman Disease

🔑 Key Principles — Nelson Section 539.2
• Diagnosis: Excisional lymph node biopsy showing histiocytes with emperipolesis (engulfed lymphocytes), S100+, CD1a-, CD68+.
• Clinical features: Massive bilateral painless cervical lymphadenopathy (classic), ± fever, weight loss, night sweats. Extranodal: skin (yellow papules), nasal cavity (obstruction), orbit (proptosis), bone (lytic lesions), CNS (mass lesions).
• Treatment: Observation for asymptomatic nodal disease (20-50% spontaneous remission). Surgery for symptomatic single-site disease. Corticosteroids, sirolimus (mTOR inhibitor), chemotherapy (methotrexate, 6-MP) for severe/extranodal. MEK inhibitors (trametinib) for MAPK pathway mutations.
• Associated conditions: Autoimmune hemolytic anemia, SLE, JIA, lymphoma (rare).

    ⚡ Reflex Prompts — Clinical Decisions in Rosai-Dorfman Disease

    📖 Summary: Rosai-Dorfman Disease — Nelson Section 539.2