Therapeutic plasma exchange for fulminant hepatic failure secondary to Wilson's disease

Shanna M Morgan, Nicole D Zantek

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

Wilson's disease (WD) is an autosomal-recessive disorder of impaired copper metabolism resulting in accumulation of copper primarily in the liver but ultimately in many organs and tissues. A small number of patients with WD initially present with fulminant hepatic failure (FHF), hypercupremia, and intravascular hemolysis. The therapeutic goals for these patients include quickly removing the copper and preparing the patient for liver transplantation. Here, we report on a 6-year-old male with WD in FHF with anemia, renal insufficiency, and coagulopathy. The patient received a series of therapeutic plasma exchanges (TPE) as adjunctive therapy to remove copper and stabilize his coagulopathy and anemia until a transplant was possible. A total of five single plasma volume (1500 mL) TPE were performed over the course of 11 days with plasma as the replacement fluid. Laboratory results demonstrated temporary improvement after each procedure. Liver transplantation was performed 12 days after beginning TPE and 35 days after admission to the hospital. TPE was a successful adjunctive therapy to bridge this patient with WD to transplantation. J. Clin. Apheresis 27:282-286, 2012.

Original languageEnglish (US)
Pages (from-to)282-286
Number of pages5
JournalJournal of Clinical Apheresis
Volume27
Issue number5
DOIs
StatePublished - Nov 1 2012

Fingerprint

Hepatolenticular Degeneration
Plasma Exchange
Acute Liver Failure
Copper
Liver Transplantation
Therapeutics
Anemia
Blood Component Removal
Plasma Volume
Hemolysis
Renal Insufficiency
Transplantation
Transplants
Liver

Keywords

  • Wilson's disease
  • copper
  • fulminant hepatic failure
  • pediatric
  • plasma exchange
  • plasmapheresis

Cite this

Therapeutic plasma exchange for fulminant hepatic failure secondary to Wilson's disease. / Morgan, Shanna M; Zantek, Nicole D.

In: Journal of Clinical Apheresis, Vol. 27, No. 5, 01.11.2012, p. 282-286.

Research output: Contribution to journalArticle

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