Liver and intrahepatic bile ducts - nontumor
Metabolic diseases
Wolman disease



Topic Completed: 1 April 2012

Revised: 23 January 2019, last major update April 2012

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PubMed Search: Wolman disease[TI]


Komal Arora, M.D.
Page views in 2018: 190
Page views in 2019 to date: 190
Cite this page: Arora K. Wolman disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/liverwolman.html. Accessed December 11th, 2019.
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
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