Acute Intravascular Hemolysis and Acute Liver Failure Associated as a First Manifestation of Wilson's Disease

摘要: In three patients, the first manifestation of Wilson's disease was a syndrome in which acute intravascular hemolysis and liver failure were associated. This developed periods; first, lasting 3 to 14 days, characterized by fatigue, fever, jaundice; second, 1 or 2 severe hemolysis; third, 6 hepatic encephalopathy. All patients died from 7 21 days after onset syndrome. The association live is characteristic disease; it rarely associated with other diseases. might result cell necrosis due accumulation copper, consequences being destruction erythrocytes large amounts copper released necrotic cells plasma.