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Primary hypereosinophilic syndrome

Disease definition

A rare hypereosinophilic syndrome characterized by hypereosinophilia produced by clonal eosinophils derived from neoplastic stem cells in the absence of any secondary cause of eosinophilia and persisting for at least six months. The condition is associated with signs of organ infiltration, dysfunction, and damage. Clinical manifestations are highly variable, depending on the organ systems involved, and include dermatologic, pulmonary, cardiac, gastrointestinal, and cerebral manifestations, among others.

ORPHA:314950

Classification level: Disorder
  • Synonym(s):
    • Clonal hypereosinophilic syndrome
    • HES-M
    • HES-N
    • Neoplastic hypereosinophilic syndrome
    • Primary HES
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: All ages
  • ICD-10: D47.5
  • OMIM: -
  • UMLS: C5679898
  • MeSH: -
  • GARD: -
  • MedDRA: -

  A summary on this disease is available in Español (2022) Français (2022) Nederlands (2022) Português (2022)

Detailed information

General public

Guidelines

Disease review articles

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

Newborn screening

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.