Histiocytosis X

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  • i. A group of disorders including Letterer-Siwe disease, Hand-Schuller-Christian disease, and eosinophilic granuloma characterized by proliferation of histiocytes
    • 1. Litterer-Siwe – occurs before age 3 and is fatal with skin, lymph nodes, bone and liver frequently involved
    • 2. Hand-Schuller-Christian syndrome – involves mostly lungs and bones; most often begins in early childhood
    • 3. eosinophilic granuloma – occurs most commonly between 20-40 involving bone although about 20% have lung involvement
  • ii. Granulomas occur in many organs, especially the lungs and bones
  • iii. Histiocytes have oval grooved nuclei and pink cytoplasm with eosinophils frequently dispersed; histiocytes are immunoreactive for S-100
  • iv. Treatment is usually steroids for lung and liver lesions and surgical excision for bone lesions
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