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Therapeutic plasmapheresis as a bridge to liver transplantation in fulminant Wilson disease
Author(s) -
Jhang Jeffrey S.,
Schilsky Michael L.,
Lefkowitch Jay H.,
Schwartz Joseph
Publication year - 2007
Publication title -
journal of clinical apheresis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.697
H-Index - 46
eISSN - 1098-1101
pISSN - 0733-2459
DOI - 10.1002/jca.20118
Subject(s) - plasmapheresis , medicine , fulminant , liver transplantation , fulminant hepatic failure , transplantation , wilson's disease , fulminant hepatitis , renal replacement therapy , hemolytic anemia , surgery , gastroenterology , disease , immunology , hepatitis , antibody
Wilson disease is an autosomal recessive disorder of copper metabolism that leads to the accumulation of copper mainly in the liver, cornea, brain, and kidney. Rarely, Wilson disease can present as fulminant hepatic failure with direct antiglobulin test–negative hemolytic anemia and renal failure. In the absence of liver transplantation, this disease is uniformly fatal because medical therapy is ineffective. This report describes the successful use of plasmapheresis for a patient with fulminant Wilson disease as a bridge to transplantation. Five daily therapeutic plasmapheresis procedures using fresh frozen plasma as a replacement fluid were performed over 6 days. Serum copper, urinary copper excretion, and hemolysis were significantly reduced and renal function improved. The patient's clinical status improved and she remained clinically stable until a liver transplant was possible. Plasmapheresis can be a successful medical treatment in fulminant Wilson disease and should be considered as a therapeutic measure to stabilize a patient by decreasing serum copper, reducing hemolysis, and helping to prevent renal tubular injury from copper and copper complexes until liver transplantation is possible. J. Clin. Apheresis. 22:, 2007 © 2007 Wiley‐Liss, Inc.