A 25-year-old man presents with acute hepatitis, Coombs-negative hemolytic anemia, and neuropsychiatric symptoms (personality change, dysarthria). Serum ceruloplasmin is 8 mg/dL (normal 20–40). Slit-lamp examination shows Kayser-Fleischer rings. What is the pathophysiology of hemolysis in this condition?
- A Autoimmune destruction of red cells via IgG antibodies
- B Copper toxicity causing oxidative damage to red cell membranes ✓
- C Hypersplenism from portal hypertension
- D Pyruvate kinase deficiency co-existing with the primary disorder
Correct answer: B. Copper toxicity causing oxidative damage to red cell membranes
Explanation
In Wilson's disease, acute episodes of hepatic copper release lead to massive copper toxicity in red cell membranes, causing direct oxidative damage and Coombs-negative intravascular hemolysis. This is a characteristic presentation and may precede or accompany acute liver failure. The hemolysis is not immune-mediated (hence Coombs-negative). Hypersplenism causes extravascular hemolysis in chronic cirrhosis but is not the mechanism in acute Wilson's. KF rings reflect copper deposition in Descemet's membrane of the cornea.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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