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Dehydrated hereditary stomatocytosis

Disease definition

Dehydrated hereditary stomatocytosis (DHS) is a rare hemolytic anemia characterized by a decreased red cell osmotic fragility due to a defect in cation permeability, resulting in red cell dehydration and mild to moderate compensated hemolysis. Pseudohyperkalemia (loss of potassium ions from red cells on storage at room temperature) is sometimes observed.


Classification level: Disorder
  • Synonym(s):
    • Hereditary xerocytosis
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Antenatal, Neonatal
  • ICD-10: D58.8
  • OMIM: 194380  616689
  • UMLS: C0272051
  • MeSH: -
  • GARD: 5623
  • MedDRA: -

Detailed information


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