Orphanet: Hemoglobin H disease

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Hemoglobin H disease

Disease definition

Hemoglobin H (HbH) disease is a moderate to severe form of alpha-thalassemia (see this term) characterized by pronounced microcytic hypochromic hemolytic anemia.


Classification level: Subtype of disorder
  • Synonym(s):
    • Alpha-thalassemia intermedia
    • HbH disease
  • Prevalence: -
  • Inheritance: Autosomal recessive 
  • Age of onset: All ages
  • ICD-10: D56.0
  • OMIM: 613978
  • UMLS: C0002312  C1260396  C3161174
  • MeSH: -
  • GARD: -
  • MedDRA: 10063435

Detailed information

Article for general public


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