Orphanet: Hémoglobinurie paroxystique nocturne

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Paroxysmal nocturnal hemoglobinuria

Disease definition

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disorder characterized by corpuscular hemolytic anemia, bone marrow failure and frequent thrombotic events.


Classification level: Disorder
  • Synonym(s):
    • Marchiafava-Micheli disease
    • PNH
  • Prevalence: 1-9 / 100 000
  • Inheritance: -
  • Age of onset: All ages
  • ICD-10: D59.5
  • OMIM: 300818  615399
  • UMLS: C0024790
  • MeSH: D006457
  • GARD: 7337
  • MedDRA: 10034042
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