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

Abstract

In 3 patients the 1st manifestation of Wilson''s disease was a syndrome in which acute intravascular hemolysis and acute liver failure were associated. This syndrome developed in 3 periods; the 1st, lasting 3-14 days, was characterized by fatigue, fever and jaundice; the 2nd, lasting 1 or 2 days, by severe acute intravascular hemolysis; and the 3rd, lasting 2-6 days, by hepatic encephalopathy. All of the patients died from liver failure 7-21 days after the onset of the syndrome. The association of acute intravascular hemolysis and acute liver 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 Cu, the consequences being acute liver failure and destruction of erythrocytes by the large amounts of Cu released from the necrotic hepatic cells to the plasma.