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Classification and external resources
ICD-10 9 MedlinePlus eMedicine MeSH D015614

In medicine, histiocytosis refers to an excessive number of histiocytes,[1] (tissue macrophages), and is typically used to refer to a group of rare diseases which share this as a characteristic. Occasionally and confusingly, the term "histiocytosis" is sometimes used to refer to individual diseases.

According to the Histiocytosis Association, 1 in 200,000 children in the United States are born with histiocytosis each year.[2] HAA also states that most of the people diagnosed with histiocytosis are children under the age of 10, although the disease can afflict adults. The University of California, San Francisco, states that the disease usually occurs from birth to age 15.[3]

Histiocytosis (and malignant histiocytosis) are both important in veterinary as well as human pathology.

Classification, and relationships to other conditions

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.[4][5] However, the classifications in ICD10 and MeSH are slightly different, as shown below:

Langerhans cell histiocytosis (LCH) I 76.0 Langerhans-cell histiocytosis
Juvenile xanthogranuloma (JXG) II 76.3 non-Langerhans-cell histiocytosis
Hemophagocytic lymphohistiocytosis (HLH) II 76.1 non-Langerhans-cell histiocytosis
Niemann–Pick disease II 75.2 non-Langerhans-cell histiocytosis
Sea-blue histiocytosis II - non-Langerhans-cell histiocytosis
Acute monocytic leukemia III 93.0 malignant histiocytic disorders
Malignant histiocytosis III 96.1 malignant histiocytic disorders
Erdheim–Chester disease II 96.1 malignant histiocytic disorders

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

Alternatively, histiocytoses may be divided into the following groups:[6]:714–724

Common treatments


Patients and families can gain support and educational materials from the ECD Global Alliance.

The clinical trials and treatment plans.


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