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

3 subordinate codes

• 2B31.20Langerhans cell histiocytosis involving the skin
• 4B4YOther specified diseases of the immune system
• 4B4ZDiseases of the immune system, unspecified

Nearby in Histiocytic or dendritic cell neoplasms

• 2B31.0Juvenile xanthogranuloma
• 2B31.1Histiocytic sarcoma
• 2B31.3Langerhans cell sarcoma
• 2B31.4Interdigitating dendritic cell sarcoma
• 2B31.5Follicular dendritic cell sarcoma
• 2B31.6Indeterminate cell histiocytosis
• 2B31.7Fibroblastic reticular cell tumour
• 4B4YOther specified diseases of the immune system