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 HLH NCIT:C34792 familial erythrophagocytic lymphohistiocytosis MONDO:0015540 SCTID:234437005 MedDRA:10058125 FHL ICD9:288.8 familial histiocytic reticulosis 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). hemophagocytic lymphohistiocytosis NCIT:C35439 familial hemophagocytic lymphohistiocytosis MEDGEN:854411 hemophagocytic syndrome Hemophagocytic Lymphohistiocytosis