raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:168956

Hypereosinophilic syndrome

Synonyms: HES

Type
Clinical group
Group
Group of disorders
Inheritance
Not applicable, Unknown
Age of onset
All ages
Status
active

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.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enHypereosinophilic syndromeHES
frSyndrome hyperéosinophilique= en
deHypereosinophile Syndrome
esSíndromes hipereosinofílicos
itSindrome da ipereosinofilia
nlHypereosinofiel syndroom
ptSíndromes hipereosinofílicos
plZespół hipereozynofilowy= en
csHypereozinofilní syndrom= en
ukГіпереозинофільний синдромГЕС
zh嗜酸性粒细胞增多综合征= en
trHipereozinofilik sendrom= en

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
MONDO0015691Evalidated
MONDO15691Evalidated
MeSHD017681Evalidated
UMLSC1540912Evalidated
GARD2804Evalidated
MedDRA10048643Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (2):

Children (3):

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

4 records
TypeClassValueAreaQualificationValidationSource
Annual incidence<1 / 1 000 0000.027 / 100 000United StatesValue and classValidated20639012[PMID]
Point prevalenceUnknown—EuropeClass onlyValidatedORPHANET
Point prevalence1-9 / 100 0003.3 / 100 000United StatesValue and classValidated20639012[PMID]_[EXPERT]
Annual incidence<1 / 1 000 000—EuropeClass onlyValidatedRARECARE surveillance of rare cancers in Europe[OTHER]

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card