Wilson disease (hepatolenticular degeneration) results from ATP7B mutation causing copper accumulation. In the liver, the pathological progression includes steatosis, hepatitis, and cirrhosis. Which histochemical stain demonstrates hepatic copper deposition, and what is the primary intracellular compartment where excess copper accumulates?
- A Rhodanine or orcein stain; lysosomes (initially as copper-metallothionein complexes) ✓
- B Prussian blue stain; mitochondria
- C PAS stain after diastase digestion; rough ER
- D Sudan black stain; cytoplasmic lipid droplets
Explanation
Copper in Wilson disease initially accumulates in hepatocyte lysosomes bound to metallothionein. Special stains for copper include rhodanine (orange-red granules) and orcein (demonstrates copper-associated protein). As the disease progresses, lysosomal storage capacity is overwhelmed and copper escapes into cytoplasm and nucleus, causing oxidative injury. Paradoxically, liver copper by rhodanine stain may be unevenly distributed and can appear negative in acute fulminant Wilson disease due to necrosis releasing copper. Iron stain (Prussian blue) detects iron; PAS-D detects α1-antitrypsin globules; Sudan black detects lipid.
Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.
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