Histiocytosis classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Classification

There are competing systems for classifying histiocytoses. According to the 1999 classification proposed by the World Health Organization, they can be divided into three categories.[1]. However, the classifications in ICD10 and MeSH are slightly different, as shown below:

Name WHO ICD10 MeSH
Langerhans cell histiocytosis (LCH) I D76.0 Langerhans-cell histiocytosis
Juvenile xanthogranuloma (JXG) II D76.3 non-Langerhans-cell histiocytosis
Hemophagocytic lymphohistiocytosis (HLH) II D76.1 non-Langerhans-cell histiocytosis
Niemann-Pick disease - E75.2 non-Langerhans-cell histiocytosis
Sea-blue histiocyte syndrome - - non-Langerhans-cell histiocytosis
Acute monocytic leukemia III C93.0 malignant histiocytic disorders
Malignant histiocytosis III C96.1 malignant histiocytic disorders
Erdheim-Chester disease - C96.1 malignant histiocytic disorders

Types of LCH have also been known as "Eosinophilic Granuloma", "Hand-Schuller-Christian Disease", "Letterer-Siwe Disease", and "Histiocytosis X". (See LCH history for details).

References

  1. Harris N, Jaffe E, Diebold J, Flandrin G, Muller-Hermelink H, Vardiman J, Lister T, Bloomfield C (1999). "The World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues. Report of the Clinical Advisory Committee meeting, Airlie House, Virginia, November, 1997". Ann Oncol. 10 (12): 1419–32. PMID 10643532.

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