{"orpha":168956,"name":"Hypereosinophilic syndrome","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":["HES"],"definition":"Hypereosinophilic syndrome (HES) constitutes a rare and heterogeneous group of disorders, defined as persistent and marked blood eosinophilia and/or tissue eosinophilia associated with a wide range of clinical manifestations reflecting eosinophil-induced tissue/organ damage.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-168956/","orphanet_url":"https://www.orpha.net/en/disease/detail/168956","names":{"fr":{"name":"Syndrome hyperéosinophilique","synonyms":["HES"],"definition":"Groupe de maladies rares et hétérogènes, définies par une éosinophilie sanguine et/ou tissulaire persistante et marquée, associée à de nombreux signes cliniques, secondaires à des lésions tissulaires/organiques induites par l'éosinophilie."},"de":{"name":"Hypereosinophile Syndrome","definition":"Die Hypereosinophilen Syndrome (HES) sind eine seltene, heterogene Gruppe von Krankheiten, die als persistierende und ausgeprägte Bluteosinophilie und/oder Gewebeosinophilie in Verbindung mit einem breiten Spektrum klinischer Manifestationen definiert sind, die als Folge eosinophiler Gewebe-/Organschädigung auftreten."},"es":{"name":"Síndromes hipereosinofílicos","definition":"Se denomina síndrome hipereosinofílico (SHE) a un conjunto de trastornos poco frecuentes y heterogéneos, caracterizados por intensa y persistente eosinofilia en sangre periférica y/o presencia de abundantes eosinófilos en los tejidos acompañada de una amplia gama de manifestaciones clínicas debidas a la lesión orgánica producida por el exceso de eosinófilos en los tejidos."},"it":{"name":"Sindrome da ipereosinofilia","definition":"La sindrome da ipereosinofila (HES) comprende un gruppo eterogeneo di malattie rare, caratterizzate da eosinofilia grave e persistente nel sangue e/o nei tessuti, associate a un'ampia gamma di segni clinici, secondari al danno indotto dall'eosinofilia ai tessuti/organi."},"nl":{"name":"Hypereosinofiel syndroom","definition":"Hypereosinofiel syndroom (HES) vormt een zeldzame en heterogene groep van stoornissen die worden gedefinieerd als aanhoudende en duidelijke eosinofilie in bloed en/of weefsel, geassocieerd met een brede waaier van klinische manifestaties, die de weefsel-/orgaanschade geïnduceerd door eosinofielen weerspiegelen."},"pt":{"name":"Síndromes hipereosinofílicos"},"pl":{"name":"Zespół hipereozynofilowy","synonyms":["HES"]},"cs":{"name":"Hypereozinofilní syndrom","synonyms":["HES"]},"uk":{"name":"Гіпереозинофільний синдром","synonyms":["ГЕС"],"definition":"Hypereosinophilic syndrome (HES) constitutes a rare and heterogeneous group of disorders, defined as persistent and marked blood eosinophilia and/or tissue eosinophilia associated with a wide range of clinical manifestations reflecting eosinophil-induced tissue/organ damage."},"zh":{"name":"嗜酸性粒细胞增多综合征","synonyms":["HES"],"definition":"嗜酸性粒细胞增多综合征（HES）是一组罕见的以血嗜酸性粒细胞持续显著增多和（或）组织中嗜酸性粒细胞浸润为特征的异质性疾病，该病临床表现多样，反映嗜酸性粒细胞增多导致的组织/器官损害。"},"tr":{"name":"Hipereozinofilik sendrom","synonyms":["HES"]}},"xrefs":[{"source":"MONDO","code":"0015691","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"15691","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D017681","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1540912","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"2804","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10048643","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97992,"name":"Rare hematologic disease"},"parents":[{"orpha":98274,"name":"Myeloproliferative neoplasm","classifications":[194,202,233]},{"orpha":217720,"name":"Non-familial restrictive cardiomyopathy","classifications":[146]}],"children":[{"orpha":3260,"name":"Idiopathic hypereosinophilic syndrome","type":"Disease"},{"orpha":314950,"name":"Primary hypereosinophilic syndrome","type":"Disease"},{"orpha":314962,"name":"Secondary hypereosinophilic syndrome","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable","Unknown"],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.027,"area":"United States","status":"Validated","source":"20639012[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Europe","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":3.3,"area":"United States","status":"Validated","source":"20639012[PMID]_[EXPERT]"},{"type":"Annual incidence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}