{"orpha":314962,"name":"Secondary hypereosinophilic syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["HES-R","Reactive hypereosinophilic syndrome","Secondary HES"],"definition":"A rare hypereosinophilic syndrome characterized by hypereosinophilia produced by reactive/non-clonal eosinophils secondary to an underlying medical condition and persisting for at least six months. The disorder can derive from non-neoplastic conditions (such as chronic infections and infestations, allergic reactions, intoxications, or autoimmune and chronic inflammatory disorders) or from neoplasms including non-myeloid malignancies, among others. It is associated with signs of organ infiltration, dysfunction, and damage. Clinical manifestations are highly variable, depending on the organ systems involved, and most commonly include dermatologic, pulmonary, cardiac, gastrointestinal, and cerebral manifestations.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-314962/","orphanet_url":"https://www.orpha.net/en/disease/detail/314962","names":{"fr":{"name":"Syndrome hyperéosinophilique secondaire","synonyms":["SHE-R","Syndrome hyperéosinophilique réactif"],"definition":"Syndrome hyperéosinophilique rare caractérisé par une hyperéosinophilie produite par des éosinophiles réactifs/non clonaux secondaire à une maladie sous-jacente et persistant pendant au moins six mois. Le syndrome peut être une manifestation d'atteintes non néoplasiques (telles que des infections et infestations chroniques, des réactions allergiques, des intoxications ou des troubles auto-immuns et inflammatoires chroniques) ou de néoplasmes (tumeurs malignes non myéloïdes comprises, entre autres). Il est associé à des signes d'infiltration, de dysfonctionnement et d'endommagement des organes. En fonction des organes atteints, les manifestations cliniques sont très inconstantes et comprennent le plus souvent des manifestations dermatologiques, pulmonaires, cardiaques, gastro-intestinales et cérébrales."},"de":{"name":"Hypereosinophiles Syndrom, sekundäres","synonyms":["HES, sekundäres","HESR","Hypereosinophiles Syndrom, reaktives"],"definition":"Eine seltene hämatologische Erkrankung, die durch eine Hypereosinophilie gekennzeichnet ist, die durch reaktive/nichtklonale Eosinophile sekundär zu einer Grunderkrankung verursacht wird und mindestens sechs Monate anhält. Die Störung kann durch nicht-neoplastische Erkrankungen (wie chronische Infektionen und Befall, allergische Reaktionen, Vergiftungen oder Autoimmunerkrankungen und chronische Entzündungen) oder durch Neoplasien, einschließlich nicht-myeloischer Malignome, verursacht werden. Sie geht mit Anzeichen von Organinfiltration, Funktionsstörungen und Läsionen einher. Die klinischen Manifestationen sind je nach betroffenem Organsystem sehr unterschiedlich und umfassen in der Regel dermatologische, pulmonale, kardiale, gastrointestinale und zerebrale Manifestationen."},"es":{"name":"Síndrome hipereosinofílico secundario","synonyms":["HES secundario","HESR","Síndrome hipereosinofílico reactivo"],"definition":"Es un síndrome hipereosinofílico poco frecuente caracterizado por hipereosinofilia producida por eosinófilos reactivos/ no clonales secundaria a una afección médica subyacente y que persiste durante al menos seis meses. El trastorno puede derivar de condiciones no neoplásicas (tales como infecciones e infestaciones crónicas, reacciones alérgicas, intoxicaciones o trastornos inflamatorios crónicos y autoinmunes) o de neoplasias, incluyendo neoplasias malignas no mieloides, entre otras. Se asocia a signos de infiltración, disfunción y daño orgánico. Las manifestaciones clínicas son muy variables, dependiendo de los sistemas orgánicos implicados, incluyendo manifestaciones dermatológicas, pulmonares, cardíacas, gastrointestinales y cerebrales con mayor frecuencia."},"it":{"name":"Sindrome ipereosinofila secondaria","synonyms":["HES secondaria","HESR","Sindrome ipereosinofila reattiva"],"definition":"Si tratta di una sindrome ipereosinofila rara, caratterizzata da ipereosinofilia causata da eosinofili reattivi/non clonali, secondaria ad una malattia preesistente, che persiste per almeno sei mesi. Può essere causata da malattie non neoplastiche (ad es. infezioni e infestazioni croniche, reazioni allergiche, intossicazioni o malattie autoimmuni e infiammatorie croniche) o da neoplasie, comprese le neoplasie non mieloidi. Si associa all'infiltrazione, alla disfunzione e al danno d'organo. I segni clinici sono molto variabili, e dipendono dagli organi coinvolti; spesso comprendono sintomi cutanei, polmonari, cardiaci, gastrointestinali e cerebrali."},"nl":{"name":"Secundair hypereosinofiel syndroom","synonyms":["HESR","Reactief hypereosinofiel syndroom","Secundaire HES"],"definition":"Een zeldzaam hypereosinofiel syndroom, gekenmerkt door hypereosinofilie geproduceerd door reactieve/niet-klonale eosinofielen secundair aan een onderliggende medische aandoening en met een duur van minstens zes maanden. De aandoening kan afgeleid zijn van niet-neoplastische aandoeningen (zoals chronische infectie en infestatie, allergische reacties, intoxicaties, of auto-immune en chronische inflammatoire aandoeningen) of van neoplasmata waaronder niet-myeloïde maligniteiten. De aandoening is geassocieerd met verschijnselen van infiltratie, disfunctie, en beschadiging van organen. Klinische manifestaties zijn zeer variabel, afhankelijk van het betrokken orgaansysteem, en omvatten doorgaans dermatologische, pulmonale, cardiale, gastro-intestinale, en cerebrale manifestaties."},"pt":{"name":"Síndrome hipereosinofílica secundária","synonyms":["HES secundária","HES-R","Síndrome hipereosinofílica reativa"],"definition":"Síndrome hipereosinofílica rara caracterizada por hipereosinofilia produzida por eosinófilos reativos/não clonais secundária a uma comorbilidade clínica subjacente e que persiste por um período não inferior a seis meses. A doença pode resultar de patologias não neoplásicas (como infeções crónicas, reações alérgicas, intoxicações ou distúrbios autoimunes e inflamatórios crónicos) ou de neoplasias, incluindo malignidades não mieloides, entre outras. Está associada a sinais infiltrativos, disfunção e danos nos órgãos. As manifestações clínicas são altamente variáveis, dependendo dos sistemas e órgãos envolvidos, e habitualmente incluem manifestações dermatológicas, pulmonares, cardíacas, gastrointestinais e cerebrais."},"pl":{"name":"Wtórny zespół hipereozynofilowy","synonyms":["HES-R","Reaktywny zespół hipereozynofilowy","Wtórny HES"]},"cs":{"name":"Sekundární hypereozinofilní syndrom","synonyms":["HES-R","Reaktivní hypereozinofilní syndrom","Sekundární HES"]},"uk":{"name":"Вторинний гіпереозинофільний синдром","synonyms":["HES-R","Реактивний гіпереозинофільний синдром","Вторинна HES"],"definition":"A rare hypereosinophilic syndrome characterized by hypereosinophilia produced by reactive/non-clonal eosinophils secondary to an underlying medical condition and persisting for at least six months. The disorder can derive from non-neoplastic conditions (such as chronic infections and infestations, allergic reactions, intoxications, or autoimmune and chronic inflammatory disorders) or from neoplasms including non-myeloid malignancies, among others. It is associated with signs of organ infiltration, dysfunction, and damage. Clinical manifestations are highly variable, depending on the organ systems involved, and most commonly include dermatologic, pulmonary, cardiac, gastrointestinal, and cerebral manifestations."},"zh":{"name":"继发性嗜酸性粒细胞增多综合征","synonyms":["反应性嗜酸性粒细胞增多综合征"]},"tr":{"name":"Sekonder hipereozinofilik sendrom","synonyms":["HES-R","Sekonder HES","Reaktif hipereozinofilik sendrom"]}},"xrefs":[{"source":"ICD-10","code":"D47.5","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0017834","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5679897","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97992,"name":"Rare hematologic disease"},"parents":[{"orpha":168956,"name":"Hypereosinophilic syndrome","classifications":[146,194,202,233]}],"children":[{"orpha":314970,"name":"Lymphocytic hypereosinophilic syndrome","type":"Clinical subtype"}],"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":null}