Histiocytosis

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**Malignant histiocytosis**
*Classifications and external resources*
ICD-10	C 96.1
ICD-9	202.3

Though histiocytosis can refer to any of several specific diseases, the term is generally used to refer to a rare blood disease that is caused by an excess of white blood cells called histiocytes. The histiocytes cluster together and can attack the skin, bones, lung, liver, spleen, gums, ears, eyes, and/or the central nervous system. The disease can range from limited involvement that spontaneously regresses to progressive multiorgan involvement that can be chronic and debilitating. In some cases, the disease can be life-threatening.

In some ways, histiocytosis is similar to cancer and has historically been treated by oncologists with chemotherapy and radiation. Unlike cancer, histiocytosis sometimes goes into remission without treatment.

The vast majority of people diagnosed with histiocytosis are children under the age of 10, but it is also found in adults of all ages.

It is estimated that histiocytosis affects 1 in 200,000 children born each year in the United States. This illness is so rare, there is little research into its cause and treatment, and it is often referred to as an orphan disease, meaning it strikes too few people to generate government-supported research and that no particular medical specialisms "own" the disease and no large organisations are providing support.

Patients and families can gain some support by contacting patient organisations such as the Histiocytosis Association of America ([1]) or the Histiocytosis Research Trust ([2]).

The Histiocytosis Association of America has several stable and proven treatment protocols available only for physicians ([3]).

Another histiocytic disorder is HLH (hemophagocytic lymphohistiocytosis), where there is an excess of both histiocytes and lymphocytes. This disease can be hereditary. Other histiocytic disorders include malignant histiocytosis and sinus histiocytosis.

Histiocytosis is frequently associated with diabetes insipidus, even after several years of diagnosis and successful therapy.

1 Alternative Names
2 Common treatments
3 See also
4 External links

[edit] Alternative Names

Pulmonary Histiocytosis X, Histiocytosis X, Eosinophilic Granuloma, Nonlipid Reticuloendotheliosis, Langerhans cell histiocytosis, Letterer-Siwe Disease, Hand-Schuller-Christian Disease, Pulmonary Langerhans Granulomatosis, LCH.