{"orpha":158048,"name":"Hemophagocytic syndrome associated with an infection","type":"Particular clinical situation in a disease or syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["IAHS","VAHS","Virus-associated hemophagocytic syndrome"],"definition":null,"note":null,"url":"https://raremap.alftech.space/d/ORPHA-158048/","orphanet_url":"https://www.orpha.net/en/disease/detail/158048","names":{"fr":{"name":"Syndrome hémophagocytaire associé à une infection","synonyms":["Hémophagocytaire, syndrome, viro-induit","IAHS","Syndrome hémophagocytaire viro-induit","VAHS"]},"de":{"name":"Hämophagozytisches Syndrom, infektionsbedingtes","synonyms":["Hämophagozytisches Syndrom, Virus-assoziiertes","IAHS","VAHS"]},"es":{"name":"Síndrome hemofagocítico asociado a infección","synonyms":["Síndrome hemofagocítico asociado a virus","SHAI","SHAV"]},"it":{"name":"Sindrome emofagocitica associata a infezione","synonyms":["IAHS","Sindrome emofagocitica associata a virus","VAHS"]},"nl":{"name":"Hemofagocytair syndroom geassocieerd met infectie","synonyms":["IAHS","VAHS","Virusgeassocieerde hemofagocytis-syndroom"]},"pt":{"name":"Síndrome hemofagocítico associado a infecção"},"pl":{"name":"Zespół hemofagocytarny towarzyszący infekcji","synonyms":["IAHS","VAHS","Zespół hemofagocytarny towarzyszący infekcji wirusowej"]},"cs":{"name":"Hemofagocytující syndrom související s infekcí","synonyms":["IAHS","VASH","Sekundární hemofagocytující syndrom"]},"uk":{"name":"Гемофагоцитарний синдром, повязаний з інфекцією","synonyms":["IAHS","VAHS","Вірус-асоційований гемофагоцитарний синдром"]},"zh":{"name":"感染相关噬血细胞综合征","synonyms":["病毒相关性噬血细胞综合征"]},"tr":{"name":"Bir enfeksiyonla ilişkili hemofagositik sendrom","synonyms":["IAHS","VAHS","Virüs-ilişkili hemofagositik sendrom"]}},"xrefs":[{"source":"ICD-10","code":"D76.2","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"3B62.5","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"778191161"},{"source":"UMLS","code":"C5207031","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7857","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98004,"name":"Rare immune disease"},"parents":[{"orpha":158041,"name":"Secondary hemophagocytic lymphohistiocytosis","classifications":[195]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-11-24","source":"31339233[PMID]_32560584[PMID]","terms":[{"hpo":"HP:0012156","label":"Hemophagocytosis","frequency":"Obligate (100%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0025540","label":"Abnormal T cell subset distribution","frequency":"Very frequent (99-80%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0031406","label":"Abnormal cytokine signaling","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012647","label":"Abnormal inflammatory response","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Very frequent (99-80%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0001876","label":"Pancytopenia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0032169","label":"Severe infection","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0031691","label":"Severe viral infection","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001744","label":"Splenomegaly","frequency":"Very frequent (99-80%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0040089","label":"Abnormal natural killer cell count","frequency":"Frequent (79-30%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0000707","label":"Abnormality of the nervous system","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001903","label":"Anemia","frequency":"Frequent (79-30%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0032204","label":"Chronic active Epstein-Barr virus infection","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001875","label":"Decreased total neutrophil count","frequency":"Frequent (79-30%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0005521","label":"Disseminated intravascular coagulation","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002910","label":"Elevated circulating hepatic transaminase concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002240","label":"Hepatomegaly","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002155","label":"Hypertriglyceridemia","frequency":"Frequent (79-30%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0011900","label":"Hypofibrinogenemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003281","label":"Increased circulating ferritin concentration","frequency":"Frequent (79-30%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0001873","label":"Thrombocytopenia","frequency":"Frequent (79-30%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0001251","label":"Ataxia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012229","label":"CSF pleocytosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006824","label":"Cranial nerve paralysis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001260","label":"Dysarthria","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002152","label":"Hyperproteinemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0020101","label":"Invasive fungal infection","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031179","label":"Nuchal rigidity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032260","label":"Opportunistic bacterial infection","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030891","label":"Periventricular white matter hyperintensities","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001250","label":"Seizure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031692","label":"Severe cytomegalovirus infection","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001298","label":"Encephalopathy","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0032256","label":"Histoplasmosis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0031700","label":"Invasive parasitic infection","frequency":"Very rare (<4-1%)","criteria":null}]}}