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Polyostotic sclerosing histiocytosis

MedGen UID:
163902
Concept ID:
C0878675
Disease or Syndrome
Synonyms: Erdheim Chester Disease; Erdheim-Chester Disease; Granulomatosis, Lipid; Lipid Granulomatosis
Modes of inheritance:
not inherited
MedGen UID:
832438
Concept ID:
CN227390
Intellectual Product
Source: Orphanet
Describes a disorder that is not inherited.
not inherited (Orphanet)
SNOMED CT: Polyostotic sclerosing histiocytosis (699537002); Erdheim-Chester disease (699537002); Erdheim-Chester syndrome (699537002); Erdheim-Chester disease (703711007)
 
Orphanet: ORPHA35687

Definition

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. [from MeSH]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPolyostotic sclerosing histiocytosis
Follow this link to review classifications for Polyostotic sclerosing histiocytosis in Orphanet.

Recent clinical studies

Prognosis

Srivanitchapoom P, Sathornsumetee S
Neurology 2015 Sep 8;85(10):e79-80. doi: 10.1212/WNL.0000000000001911. PMID: 26346832

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