Hepatocyte inclusions of alpha 1-antichymotrypsin in a patient with partial deficiency of alpha 1-antichymotrypsin and chronic liver disease

Histopathology. 1990 Mar;16(3):221-5. doi: 10.1111/j.1365-2559.1990.tb01107.x.

Abstract

We present a case of chronic liver disease with selective and exclusive hepatocyte endoplasmic reticulum storage of alpha 1-antichymotrypsin in the form of granules, detected by specific immunohistochemistry at the light microscopy level and corresponding to material found in dilated endoplasmic reticulum of hepatocytes by electron microscopy. The patient had intermediate deficiency of alpha 1-antichymotrypsin. Thus, the hepatocyte accumulation of alpha 1-antichymotrypsin may indicate the presence of an export block resembling that of a closely-related protein, namely PiZ alpha 1-antitrypsin. It is proposed that hepatocyte storage of alpha 1-antichymotrypsin may be an expression of an inborn error of metabolism bearing the characteristics of endoplasmic reticulum storage diseases such as PiZ alpha 1-antitrypsin deficiency and hereditary hypofibrinogenaemia.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Chronic Disease
  • Endoplasmic Reticulum / metabolism*
  • Endoplasmic Reticulum / ultrastructure
  • Female
  • Humans
  • Immunohistochemistry
  • Liver Diseases / metabolism*
  • Liver Diseases / pathology
  • Middle Aged
  • alpha 1-Antichymotrypsin / deficiency*
  • alpha 1-Antichymotrypsin / metabolism

Substances

  • alpha 1-Antichymotrypsin