{"orpha":158038,"name":"Primary hemophagocytic lymphohistiocytosis","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":["Genetic HLH","Genetic hemophagocytic lymphohistiocytosis","Primary HLH"],"definition":null,"note":"This term does not characterize a disease but a group of diseases. To learn about the diseases included under this term, you can consult the classifications.","url":"https://raremap.alftech.space/d/ORPHA-158038/","orphanet_url":"https://www.orpha.net/en/disease/detail/158038","names":{"fr":{"name":"Lymphohistiocytose hémophagocytaire primaire","synonyms":["HLH génétique","HLH primaire","Hémophagocytose lymphohistiocytaire génétique","Hémophagocytose lymphohistiocytaire primaire","Lymphohistiocytose hémophagocytaire génétique"]},"de":{"name":"Lymphohistiozytose, hämophagozytische, primäre","synonyms":["Lymphohistiozytose, hämophagozytische, genetisch bedingte","Primäre HLH"]},"es":{"name":"Linfohistiocitosis hemofagocítica primaria","synonyms":["LHH genética","LHH primaria","Linfohistiocitosis hemofagocítica genética"]},"it":{"name":"Linfoistiocitosi emofagocitica primaria","synonyms":["HLH gentica","HLH primaria","Linfoistiocitosi emofagocitica genetica"]},"nl":{"name":"Primaire hemofagocytaire lymfohistiocytose","synonyms":["Genetische HLH","Genetische hemofagocytaire lymfohistiocytose","Primaire HLH"]},"pt":{"name":"Linfo-histiocitose hemofagocítica primária","synonyms":["HLH primária","Linfo-histiocitose hemofagocítica genética"]},"pl":{"name":"Pierwotna limfohistiocytoza hemofagocytarna","synonyms":["Genetyczna limfohistiocytoza hemofagocytarna"]},"cs":{"name":"Primární hemofagocytující lymfohistiocytóza","synonyms":["Geneticky podmíněná hemofagocytující lymfohistiocytóza"]},"uk":{"name":"Первинний гемофагоцитарний лімфогістіоцитоз","synonyms":["Генетичний гемофагоцитарний лімфогістіоцитоз","Генетичний HLH","Первинний HLH"]},"zh":{"name":"原发性噬血细胞性淋巴组织细胞增多症","synonyms":["遗传性噬血细胞性淋巴组织细胞增多症"]},"tr":{"name":"Primer hemofagositik lenfohistiyositoz","synonyms":["Genetik hemofagositik lenfohistiyositoz"]}},"xrefs":[{"source":"ICD-11","code":"4A01.23","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1523519942"}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98004,"name":"Rare immune disease"},"parents":[{"orpha":158032,"name":"Hemophagocytic syndrome","classifications":[195]},{"orpha":169361,"name":"Immune dysregulation disease with immunodeficiency","classifications":[156,195]},{"orpha":506219,"name":"Rare disorder potentially indicated for hematopoietic stem cell transplant","classifications":[233]}],"children":[{"orpha":331249,"name":"Primary hemophagocytic lymphohistiocytosis with hypopigmentation","type":"Category"},{"orpha":664482,"name":"Primary hemophagocytic lymphohistiocytosis without hypopigmentation","type":"Category"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}