A 30-year-old woman presents with acute liver failure, hemolytic anemia (Coombs-negative), neuropsychiatric symptoms, and a Kayser-Fleischer ring visible on slit-lamp. Serum ceruloplasmin is 8 mg/dL (low). Urinary copper is 1800 µg/24h. She is deeply jaundiced with INR 4.8. What is the definitive treatment?
- A D-penicillamine chelation therapy
- B Trientine + zinc therapy
- C Liver transplantation ✓
- D Plasma exchange followed by D-penicillamine
Correct answer: C. Liver transplantation
Explanation
Wilson's disease presenting as acute liver failure (ALF) with hemolysis is rapidly fatal and does not respond to chelation therapy alone in this setting — D-penicillamine in ALF can worsen the condition by mobilizing copper and is therefore contraindicated. The New Wilson Index (Nazer score) or King's College criteria are used to predict mortality. Liver transplantation is the only life-saving intervention in Wilson's ALF and is also curative for the metabolic defect, correcting hepatic copper handling. Chelation is used for non-fulminant Wilson's disease.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.