Acute intravascular hemolysis and acute liver failure associated as a first manifestation of Wilson's disease.

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