World Of Taxonomy
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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