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Evans syndrome

Disease definition

Evans syndrome is a rare chronic hematologic disorder characterized by the simultaneous or sequential association of autoimmune hemolytic anemia (AIHA; a disorder in which auto-antibodies are directed against red blood cells causing anemia of varying degrees of severity) with immune thrombocytopenic purpura (ITP; a coagulation disorder in which auto-antibodies are directed against platelets causing hemorrhagic episodes) (see these terms) and occasionally autoimmune neutropenia, in the absence of a known underlying etiology.


  • Synonym(s):
    • Autoimmune hemolytic anemia and autoimmune thrombocytopenia
    • Immune pancytopenia
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Childhood, Adult
  • ICD-10: D69.3
  • OMIM: -
  • UMLS: C0272126
  • MeSH: -
  • GARD: 6389
  • MedDRA: 10053873

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