disease has feature disease arises from structure disease arises from alteration in structure macrophage Hepatosplenomegaly Pancytopenia Lymphadenopathy syndromic disease lymphatic system disorder hemophagocytic syndrome https://github.com/monarch-initiative/mondo/issues/9097 https://rarediseases.info.nih.gov/diseases/6589/hemophagocytic-lymphohistiocytosis https://www.malacards.org/card/hemophagocytic_lymphohistiocytosis GARD:0020024 Orphanet:158032 UMLS:C3887558 NANDO:2200032 NCIT:C34792 familial erythrophagocytic lymphohistiocytosis FHL ICD9:288.8 familial histiocytic reticulosis hemophagocytic lymphohistiocytosis NCIT:C35439 hemophagocytic syndrome HLH 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 MONDO:0015540 SCTID:234437005 MedDRA:10058125 ICD10CM:D76.1 NORD:1938 DOID:0050120 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). familial hemophagocytic lymphohistiocytosis MEDGEN:854411 Hemophagocytic Lymphohistiocytosis