2B31.2Level 4
Langerhans cell histiocytosis
**Definition:** A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognised: eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schuller-Christian disease. The clinical course is generally related to the number of organs affected at presentation.
**Inclusions:** - Histiocytosis X
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