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DeCS
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Descriptor English:
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Erdheim-Chester Disease
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Descriptor Spanish:
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enfermedad de Erdheim-Chester
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Descriptor Portuguese:
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Doença de Erdheim-Chester
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Synonyms English:
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Erdheim Chester Disease
Granulomatosis, Lipid
Lipid Granulomatosis
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Tree Number:
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C15.604.250.410.224
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Definition English:
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A rare form of non-Langerhans-cell histiocytosis (HISTIOCYTOSIS, NON-LANGERHANS-CELL) with onset in middle age. The systemic disease is characterized by infiltration of lipid-laden macrophages, multinucleated giant cells, an inflammatory infiltrate of lymphocytes and histiocytes in the bone marrow, and a generalized sclerosis of the long bones. |
History Note English:
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2003
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Allowable Qualifiers English:
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Record Number:
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37288
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Unique Identifier:
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D031249
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Occurrence in VHL:
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Similar:
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DeCS
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