Life saving plasmapheresis for the management of hemolytic crisis and acute liver failure in Wilson's Disease

Mohammad Reza Pashaei, Hossein Ajdarkosh, Nasser Ebrahimi Daryani, Peiman Habibollahi, Mohammad Taghi Beigmohammadi

Research output: Contribution to journalArticle

1 Scopus citations

Abstract

Wilson's disease, caused by a deficient cellular copper export system, is transmitted as an autosomal recessive inherited disorder and results in copper accumulation in liver and other organs, particularly in brain. Acute hepatic failure and severe Coombs' negative hemolysis may occur in the course of the disease which has a poor prognosis and most patients do not survive the crisis. Only liver transplantation has been recommended as an effective medical intervention. Herein, we presented a 25-year-old woman with impaired consciousness, acute hepatic failure and hemolysis who was treated with plasmapheresis and albumin replacement. Beside improvement in medical condition, serum copper and hemolysis decreased significantly and renal function was preserved. We concluded that plasmapheresis may be a life saving intervention during fulminant hepatic failure of Wilson's disease.

Original languageEnglish (US)
Pages (from-to)154-158
Number of pages5
JournalHepatitis Monthly
Volume9
Issue number2
StatePublished - Sep 25 2009
Externally publishedYes

Keywords

  • Acute Hepatic Failure
  • Hemolysis
  • Plasmapheresis
  • Wilson's disease

ASJC Scopus subject areas

  • Hepatology
  • Infectious Diseases

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    Pashaei, M. R., Ajdarkosh, H., Daryani, N. E., Habibollahi, P., & Beigmohammadi, M. T. (2009). Life saving plasmapheresis for the management of hemolytic crisis and acute liver failure in Wilson's Disease. Hepatitis Monthly, 9(2), 154-158.