Which of the following features in liver biopsy is seen in alpha 1 antitrypsin deficiency
A. PAS positive and diastase resistant globules are seenB. Cirrhosis of liver
C. Mallory hyaline
D. Bile, duct proliferation
E. Amyloid deposition
The Answer
is. (A) PAS positive and diastase resistant globules are seen; (B)
Cirrhosis of liver ;(C) Mallory
hyaline
Alpha-1 antitripsin is a glycoprotein synthesized in
liver. This protein inhibits leukocytic and bacterial proteinase and other
proteolytic enzymes e.g. chymotrypsin, plasmin, thrombin elastase collagenase
and Hagmen factor a1 antitrypsin
deficiency is characterised by presence of round to oval cytoplasmic granular
inclusion in hepatocytes which’ in haematoxylin and eosin stains are
acidophilic. and have indistinctly demarcated cytoplasm. They are PAS positive Diastase resistance globules.
• Infrequently fatty changes and Mallory bodies are
present.
• α1 antitrypsin deficiency leads to cirrh’osis and hepatocellular carcinoma.
