Liver and intrahepatic bile ducts - nontumor
Metabolic diseases
Wolman disease

Author: Komal Arora, M.D. (see Authors page)

Revised: 14 November 2017, last major update April 2012

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Wolman disease[TI]

Cite this page: Arora, K. Wolman disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/liverwolman.html. Accessed November 20th, 2017.
Definition / general
  • Deficient activity of lysosomal acid lipase (LAL) results in massive accumulation of cholesteryl esters and triglycerides in most tissues of the body
  • Occurs in infancy and is nearly always fatal before the age of 1 year
  • Heterozygous frequency of 3% in those of Iranian Jewish ancestry in Los Angeles, California (Genet Test Mol Biomarkers 2011;15:395)
Microscopic (histologic) description
  • Foamy Kupffer cells and hepatocytes
Electron microscopy description
  • Lipid droplets in hepatocytes and Kupffer cells
  • Cholesterol clefts in Kupffer cells