A patient with Wilson's disease has corneal Kayser-Fleischer rings, liver disease, and haemolytic anaemia. The biochemical basis of haemolysis in Wilson's disease is:

• A Copper chelation depletes G6PD, causing G6PD-like haemolysis
• B Elevated serum copper inhibits haeme synthesis, causing sideroblastic anaemia
• C Copper causes complement activation at the RBC membrane, causing Coombs-positive haemolysis
• D Free copper deposits in RBCs, oxidising glutathione and membrane proteins, causing Coombs-negative haemolytic anaemia ✓

Explanation

In Wilson's disease, defective ATP7B copper-transporting ATPase causes copper accumulation. Free (non-ceruloplasmin-bound) copper acts as a pro-oxidant via Fenton-like reactions, generating hydroxyl radicals that oxidise RBC membrane lipids, proteins, and glutathione. This causes Coombs-negative (non-immune) haemolytic anaemia — an important diagnostic clue. Episodic haemolytic crises can occur before overt hepatic disease is recognised.

Reference: Harper's Illustrated Biochemistry, 32nd ed.

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