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Langerhans Cell Histiocytosis
Langerhans cell histiocytosis (LCH) is a rare spectrum of
disorders characterized by overproduction (proliferation) and
accumulation of a specific type of white blood cell (histiocyte)
in the various tissues and organs of the body (lesions). The
lesions may include certain distinctive granule-containing cells
(Langerhans cells) involved in certain immune responses, as well
as other white blood cells (e.g., monocytes, eosinophils).
Associated symptoms and findings may vary from case to case,
depending upon the specific tissues and organs affected and the
extent of involvement.
Most affected individuals have single or multiple bone lesions
characterized by degenerative changes and loss of the calcium
of bone (osteolysis). Although the skull is most commonly
affected, there may also be involvement of other bones, such as
those of the spine (vertebrae) and the long bones of the arms
and legs. Affected individuals may have no apparent symptoms
(asymptomatic), and may experience associated pain and swelling,
and/or develop certain complications, such as fractures or
secondary compression of the spinal cord. In some cases, other
tissues and organs may also be affected, including the skin,
lungs, or other areas. In some individuals, LCH may be
associated with involvement of the pituitary gland leading to
diabetes insipidus. The exact cause of Langerhans cell
histiocytosis is unknown.
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