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Langerhans cell g. see under histiocytosis.
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Langerhans cell h. a generic term embracing a group of disorders characterized by proliferation of Langerhans cells (q.v.); children are more often affected than adults. Lesions may be unifocal or multifocal and may involve the bone marrow, endocrine system, or lungs (the last being more common in adults than in children). Although the cause is uncertain, these disorders are believed to arise from disturbances in regulation of the immune system. Called also eosinophilic granuloma or granulomatosis and Langerhans cell granulomatosis. Formerly called h. X.
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Langerhans cell h., acute disseminated Letterer-Siwe disease.
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Langerhans cell h., multifocal Langerhans cell histiocytosis occurring as erosive accumulations of proliferating Langerhans' cells, commonly within the medullary cavities of bones, but also affecting the skin, gingiva, lungs, and stomach. It most commonly affects children and is accompanied by seborrheic eruptions, fever, frequent occurrences of otitis media, mastoiditis, and upper respiratory tract infection, often with lymphadenopathy and splenomegaly. When the triad of calvarial bone defects, exophthalmos, and diabetes insipidus is present, it is referred to as Hand-Schüller-Christian disease.
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Langerhans cell h., unifocal Langerhans cell histiocytosis occurring as a single osteolytic lesion, usually in a long or flat bone; it may be asymptomatic or may produce bone pain, tenderness, and swelling and, sometimes, pathologic fracture.
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