Langerhans Cell Histiocytosis (LCH) is among a rare and diverse group of disorders affecting primarily children. Although it was first described more than a century ago, much remains to be discovered about the causes of LCH. LCH was previously known as HIstiocytosis-X, with the terms eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe disease applied to various forms of the disease.
The presentation of LCH is variable. Localized disease may include “punched-out” lesions of the bones, skin rash often confused with eczema, or lymph node enlargement. The bony lesions are commonly in the flat bones such as the skull, long bones of the arms and legs, and ribs. Some patients may have chronic ear infections or discharge. Systemic disease is characterized by internal organ involvement which may include the liver, spleen, lungs, bone marrow, gastrointestinal tract or masses behind the eyes. The systemic form of LCH is seen most commonly in infants and children less than 2 years of age.
A diagnosis of LCH can only be made following a biopsy and microscopic examination of the affected tissue. To determine the extent of the disease and the best treatment plan, several other tests will be required. These may include X-rays of the chest and bones (called a bone or skeletal survey), CT scans and bone scan. Laboratory studies of the blood will be required to determine involvement of the bone marrow and liver. A bone marrow biopsy is sometimes performed.
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LCH Fast Facts