Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.
In medicine, histiocytosis is an excessive number of histiocytes,[1] that is an excessive number of tissue macrophages, and is typically used to refer to a group of rare diseases which share this as a characteristic. Occasionally and confusingly it is sometimes used to refer to individual diseases.
The histiocytes may attack skin, bone, muscles, and other important organs, including the liver, lung, spleen, and hematopoietic system. The disease is somewhat similar to cancer, and treatment often involves radiation and chemotherapy.
According to the Histiocytosis Association of America, 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. 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.
Histiocytosis is frequently associated with diabetes insipidus, even after several years of diagnosis and successful therapy.
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:
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).
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