Effective removal of copper by plasma exchange in fulminant Wilson's 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.


METHODS
The utility of therapeutic plasma exchange to rapidly remove copper and reduce toxic serum copper levels was studied in two patients with fulminant Wilson's disease.


RESULTS
Intensive plasma exchange using fresh-frozen plasma replacement removed substantial amounts of copper from the hypercupremic patients, resulting in a rapid reduction in serum copper levels and decreased hemolysis. The net copper removal was proportional to the serum level, ranging from 7,000 to 11,800 micrograms per procedure in one patient and from 3,700 to 6,800 micrograms in the other.


CONCLUSIONS
Plasma exchange allows a rapid reduction in elevated serum copper levels in patients with fulminant Wilson's disease. This leads to an amelioration of hemolytic anemia and provides clinical stabilization until liver transplantation can be performed.
Kiss
J E
JE
Division of Hematology/Bone Marrow Transplantation, University of Pittsburgh School of Medicine, Pennsylvania, USA.
Berman
D
D
Van Thiel
D
D
D000293:Adolescent; D000328:Adult; D000743:Anemia, Hemolytic / Q000209:etiology; D003300:Copper / Q000097:blood*; D005260:Female; D006501:Hepatic Encephalopathy / Q000209:etiology; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000628:therapy*; D006801:Humans; D010951:Plasma Exchange
D003300:Copper