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 ]

This is just here as a test because I lose it

Term information

database cross reference
  • NCIT:C35439 (MONDO:equivalentTo)
  • SCTID:234437005 (MONDO:equivalentTo)
  • MedDRA:10058125 (Orphanet:158032)
  • ICD10:D76.1 (DOID:0050120)
  • UMLS:C3887558 (Orphanet:158032)
  • ICD9:288.8 (i2s)
  • GARD:0006589 (MONDO:equivalentTo)
  • Orphanet:158032 (MONDO:equivalentTo)
  • UMLS:C0024291 (Orphanet:158032)
  • COHD:439789 (MONDO:equivalentTo)
  • DOID:0050120 (MONDO:equivalentTo)
  • NCIT:C34792 (MONDO:equivalentTo)
Subsets

gard_rare, ordo_group_of_disorders

abbreviation
HLH [ GARD:0006589 Orphanet:158032 ]

abbreviation
FHL [ GARD:0006589 ]

closeMatch

http://identifiers.org/mesh/D051359

http://identifiers.org/snomedct/190958003

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

definition

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).

exactMatch

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

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

http://identifiers.org/snomedct/234437005

http://identifiers.org/meddra/10058125

http://linkedlifedata.com/resource/umls/id/C0024291

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

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

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

has exact synonym

HLH

hemophagocytic syndrome

hemophagocytic lymphohistiocytosis

has related synonym

familial hemophagocytic lymphohistiocytosis

hemophagocytic disorder

familial erythrophagocytic lymphohistiocytosis

familial histiocytic reticulosis

haemophagocytic syndrome

FHL

id

MONDO:0015540

seeAlso

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