has modifier disease has feature disease arises from structure macrophage Hepatosplenomegaly Pancytopenia Lymphadenopathy syndromic disease lymphatic system disease hemophagocytic syndrome COHD:439789 DOID:0050120 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 FHL GARD:0006589 HLH HPS 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). ICD10:D76.1 ICD9:288.8 MONDO:0015540 MedDRA:10058125 NCIT:C34792 NCIT:C35439 Orphanet:158032 SCTID:234437005 UMLS:C0024291 UMLS:C3887558 familial erythrophagocytic lymphohistiocytosis familial hemophagocytic lymphohistiocytosis familial histiocytic reticulosis haemophagocytic syndrome hemophagocytic disorder hemophagocytic lymphohistiocytosis hemophagocytic syndrome https://rarediseases.info.nih.gov/diseases/6589/hemophagocytic-lymphohistiocytosis rare