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Familial Letterer-Siwe disease MeSH Supplementary Concept Data 2025


MeSH Supplementary
Familial Letterer-Siwe disease
Unique ID
C538636
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/C538636
Entry Term(s)
Histiocytosis X, acute disseminated
Registry Numbers
0
Heading Mapped to
*Histiocytosis, Langerhans-Cell
Frequency
239
Note
A hereditary form of eosinophilic granuloma that affects the liver, spleen, and lymph nodes. It is characterized by fever, distended abdomen, enlarged liver and spleen (hepatosplenomegaly); JAUNDICE; DERMATITIS; ANEMIA; NEUTROPENIA; and THROMBOCYTOPENIA. Onset is typically in the first year of life. OMIM: 246400
Date of Entry
2010/08/25
Revision Date
2016/07/11
Familial Letterer-Siwe disease Preferred
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