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Langerhans cell histiocytosis

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

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.

sections/codes in this section (2B31.2-2B31.2)

• Langerhans cell histiocytosis involving the skin  (2B31.20)
• Other specified Langerhans cell histiocytosis  (2B31.2Y)
• Langerhans cell histiocytosis, unspecified  (2B31.2Z)

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