Endoplasmic storage disease of liver: characterization of intracytoplasmic hyaline inclusions

Histopathology. 1989 Nov;15(5):473-81. doi: 10.1111/j.1365-2559.1989.tb01607.x.

Abstract

Two cases of endoplasmic storage disease of liver are described. The liver tissue in each case showed numerous intracytoplasmic hyaline inclusions of varying sizes with formation of ground-glass hepatocytes. These inclusions were pale eosinophilic in hematoxylin & eosin stained sections, and were periodic acid-Schiff and HBsAg negative. Immunoperoxidase studies revealed strong positivity for fibrinogen and complement components C3 and C4 in case 1 and exclusive positivity for fibrinogen in case 2. On electron microscopy, the inclusions appeared as granular or fibrillar material within dilated cisternae of rough endoplasmic reticulum.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Carcinoma, Hepatocellular / complications
  • Complement C3 / metabolism
  • Complement C4 / metabolism
  • Endoplasmic Reticulum / metabolism
  • Endoplasmic Reticulum / pathology*
  • Fibrinogen / metabolism
  • Hepatitis B / complications
  • Humans
  • Hyalin / metabolism*
  • Inclusion Bodies / immunology
  • Inclusion Bodies / metabolism
  • Inclusion Bodies / pathology*
  • Liver Cirrhosis / complications
  • Liver Diseases / immunology
  • Liver Diseases / metabolism
  • Liver Diseases / pathology*
  • Liver Neoplasms / complications
  • Male

Substances

  • Complement C3
  • Complement C4
  • Fibrinogen