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hemophagocytic syndrome

Go to external page http://purl.obolibrary.org/obo/MONDO_0015540

Hemophagocytic syndrome (HPS) is a rare immune disease and a potentially life-threatening disorder characterized by cytokine storm and overwhelming inflammation causing fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. It can be either primary due to a genetic defect (primary hemophagocytic lymphohistiocytosis), or secondary to malignancies, to infections, most commonly with viruses such as Epstein-Barr virus or cytomegalovirus, human immunodeficiency virus, or to autoimmune disorders such as systemic lupus erythematosus or adult-onset Still disease (secondary hemophagocytic lymphohistiocytosis). [ Orphanet:158032 ]

Synonyms: HLH Hemophagocytic Lymphohistiocytosis hemophagocytic syndrome hemophagocytic lymphohistiocytosis

This is just here as a test because I lose it

Term information

database cross reference
  • Orphanet:158032 (MONDO:equivalentTo)
  • NCIT:C34792 (MONDO:equivalentTo)
  • ICD9:288.8 (MONDO:relatedTo)
  • NORD:1938 (MONDO:NORD)
  • MEDGEN:854411 (MONDO:equivalentTo)
  • DOID:0050120 (MONDO:equivalentTo)
  • NCIT:C35439 (MONDO:equivalentTo)
  • GARD:20024 (MONDO:GARD)
  • SCTID:234437005 (MONDO:equivalentTo)
  • NANDO:2200032 (https://orcid.org/0000-0003-0011-764X)
  • UMLS:C3887558 (MONDO:equivalentTo)
  • MedDRA:10058125 (Orphanet:158032/e)
Subsets

gard_rare, otar, disease_grouping, rare, nord_rare, ordo_group_of_disorders

abbreviation
FHL [ GARD:0006589 ]

abbreviation
HLH [ Orphanet:158032 GARD:0006589 ]

closeMatch

http://identifiers.org/meddra/10058125

comment

Editor note: NCIT treats hemophagocytic syndrome and Hemophagocytic Lymphohistiocytosis as distinct; we merge here but this should be checked. See https://github.com/NCI-Thesaurus/thesaurus-obo-edition/issues/19

exactMatch

http://www.orpha.net/ORDO/Orphanet_158032

http://purl.obolibrary.org/obo/NCIT_C35439

http://identifiers.org/snomedct/234437005

http://linkedlifedata.com/resource/umls/id/C3887558

http://purl.obolibrary.org/obo/NCIT_C34792

http://purl.obolibrary.org/obo/DOID_0050120

http://identifiers.org/medgen/854411

has related synonym

hemophagocytic disorder

familial hemophagocytic lymphohistiocytosis

familial erythrophagocytic lymphohistiocytosis

familial histiocytic reticulosis

haemophagocytic syndrome

FHL

id

MONDO:0015540

seeAlso

https://rarediseases.info.nih.gov/diseases/6589/hemophagocytic-lymphohistiocytosis