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Letterer-Siwe disease
rareA multifocal, multisystem form of Langerhans-cell histiocytosis. There is involvement of multiple organ systems including the bones, skin, liver, spleen, and lymph nodes. Patients are usually infants presenting with fever, hepatosplenomegaly, lymphadenopathy, bone and skin lesions, and pancytopenia.
- Identifiers
- MONDO:0009519 OMIM:246400Orphanet:99870
- Also known as
- acute and disseminated Langerhans cell histiocytosis · acute disseminated Langerhans cell histiocytosis · multifocal multisystem Langerhans cell histiocytosis
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