d-Penicillamine in Wilson's disease presenting as acute liver failure with hemolysis

Abstract
Wilson's disease in a young woman presenting with an acute course is described. The clinical manifestations were fulminant hepatic failure associated with marked intravascular hemolysis. Immediated-penicillamine and high-dose steroid therapy did not infuence the course of the disease. Necropsy revealed an increased hepatic copper content and cirrhosis with extensive necrosis of the liver.