Alpha 1 antitrypsin liver disease differential diagnosis of PAS-positive, diastase-resistant globules in liver cells.

In 500 consecutive autopsies there were 27 cases in which the livers contained PAS-positive, diastase-resistant globules within hepatocytes. On the basis of morphologic findings and immunoperoxidase staining the inclusions were separable into two groups. There were 14 (2.8%) cases in which the globules were periportal in location and stained positively with the specific AAT immunoperoxidase method (Type 1 globules). In 13 (2.6%) cases, the globules were located in the centrilobular region of the liver or at the edge of the central ischemic zone. These globules did not stain with the specific immunoperoxidase technic (Type 2 globules). Cirrhosis was found in 10 (71%) of the 14 livers containing Type 1 globules. Dysplastic liver cells were present in four cases. No liver cell cancer was present in any of the cases. No fibrosis or cirrhosis was found in any of the 13 livers containing Type 2 globules. They were always present in the centrilobular areas and most likely were the result of sinusoidal congestion and anoxia. The immunocytochemical method is useful in separating the two types of PAS-positive, diastase-resistant globules. Type 1 inclusions are associated with alpha 1 antitrypsin deficiency.