Orphanet: Paroxysmal cold hemoglobinuria

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Paroxysmal cold hemoglobinuria

Disease definition

Paroxysmal cold hemoglobinuria (PCH) is a very rare subtype of autoimmune hemolytic anemia (AIHA, see this term), caused by the presence of cold-reacting autoantibodies in the blood and characterized by the sudden presence of hemoglobinuria, typically after exposure to cold temperatures.


Classification level: Disorder
  • Synonym(s):
    • Donath-Landsteiner hemolytic anemia
    • Donath-Landsteiner syndrome
    • PCH
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Multigenic/multifactorial 
  • Age of onset: Childhood
  • ICD-10: D59.6
  • OMIM: -
  • UMLS: C0086774  C0272129
  • MeSH: C538618
  • GARD: 7335
  • MedDRA: -

Detailed information


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