EFFECTIVE REMOVAL OF COPPER BY PLASMA-EXCHANGE IN FULMINANT WILSONS-DISEASE

BACKGROUND: Patients who present with fulminant hepatic failure due to Wilson's disease may develop hemolytic anemia and renal insufficiency In this entity, acute hepatocellular necrosis triggers the release of copper ions into the circulation; which leads to toxic effects on red cell metabolic pathways and hemolysis. STUDY DESIGN AND METHODS: The utility of therapeutic-plasma exchange to rapidly remove,copper and re duce toxic serum copper levels was studied in two patients with fulmin ant Wilson's disease. RESULTS: Intensive plasma exchange using fresh-f rozen plasma replacement removed substantial amounts of copper from th e hypercupremic patients, resulting in a rapid reduction in serum copp er levels and decreased hemolysis. The net copper removal was proporti onal to the serum level, ranging from 7,000 to 11,800 mu g per procedu re in one patient and from 3,700 to 6,800 mu g in the other. CONCLUSIO N: Plasma exchange allows a rapid reduction in elevated serum copper l evels in patients with fulminant Wilson's disease. This leads to an am elioration of hemolytic anemia end provides clinical stabilization unt il liver transplantation can be performed.