A 31-year-old man has progressive dyspnea with forced expiratory volume reduced out of proportion to his age. Chest imaging shows basal panacinar emphysema. He also has transaminases two times the upper limit of normal. Liver biopsy would be expected to show:

• A Ground-glass hepatocytes positive for HBsAg
• B Perisinusoidal iron deposition with intact Kupffer cells
• C Copper granules on rhodanine stain in periseptal hepatocytes
• D PAS-positive, diastase-resistant globules in periportal hepatocytes ✓

Explanation

Alpha-1 antitrypsin deficiency (PiZZ genotype) causes misfolded polymerized D1AT to accumulate in hepatocyte endoplasmic reticulum, visible as PAS-positive, diastase-resistant globules concentrated in periportal hepatocytes. Lung disease arises from unopposed neutrophil elastase activity because circulating antiprotease levels are low, producing basal panacinar emphysema. Iron deposition with preserved Kupffer cell sparing suggests hereditary hemochromatosis, copper accumulation indicates Wilson disease, and ground-glass cells are seen in chronic hepatitis B.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.