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Alternate Names: LCH, Hand-Schuller-Christian syndrome, Letterer-Siwe disease, eosinophilic granuloma
Langerhans Cell Histiocytosis (LCH) is a rare, proliferative disorder of dendritic cells. Dendritic cells are a type of immune cell that can be found in various tissues in the body. In LCH, the dendritic cells (also called Langerhans cells or histiocytes) multiply and cause damage to the tissue where they are located. The clinical picture of LCH can vary widely. Often, it involves only a single organ such as skin, bone, or lymph nodes. Other patients can present with multi-system disease involving any organ of the body, most commonly bone marrow, lungs, liver and brain. While LCH is not a cancer, it typically requires treatment with chemotherapy similar to that used to treat many childhood cancers.
There is no known associated risk-factor for pediatric LCH, although research has suggested infectious and environmental triggers for the disease. Cigarette smoking is clearly linked with increased risk for pulmonary LCH in adults.
Patients with only skin involvement will occasionally have improvement without therapy. Single bone lesions can often be cured with surgical removal followed by observation. More extensive disease requires treatment with chemotherapy for a duration of about 1 year.
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