Langerhans cell histiocytosis

Description

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

Exclusions from above levels

2E03 – Malignant neoplasm metastasis in bone or bone marrow
http://id.who.int/icd/entity/501778925 – Malignant neoplasm metastasis in lymph nodes
http://id.who.int/icd/entity/97145147 – Malignant neoplasm metastasis in thymus gland

Child codes

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

Sibling codes

2B31.0 – Juvenile xanthogranuloma
2B31.1 – Histiocytic sarcoma
2B31.3 – Langerhans cell sarcoma
2B31.4 – Interdigitating dendritic cell sarcoma
2B31.5 – Follicular dendritic cell sarcoma
2B31.6 – Indeterminate cell histiocytosis
2B31.7 – Fibroblastic reticular cell tumour
2B31.Y – Other specified histiocytic or dendritic cell neoplasms
2B31.Z – Histiocytic or dendritic cell neoplasms, unspecified

Foundation entity

Langerhans cell histiocytosis – http://id.who.int/icd/entity/216625985

Allowed postcoordination axes

Part of: 2B31: Histiocytic or dendritic cell neoplasms › 02: Neoplasms