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Evans syndrome

ORPHA:1959· ICD-10 D69.3

Definition

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) and occasionally autoimmune neutropenia, in the absence of a known underlying etiology.

Prevalence
1-9 / 1 000 000
Inheritance
Not applicable
Age of onset
Adult, Childhood