Glycogenic Hepatopathy

Published on 21/04/2017 by admin

Filed under Pathology

Last modified 21/04/2017

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 Pale, enlarged hepatocytes may be mistaken for normal or interpreted as fixation artifact

• Fatty liver disease

image Pale, enlarged hepatocytes may be misinterpreted as ballooning hepatocyte degeneration
• Glycogen storage disease

image No history of diabetes mellitus
image
Pale Swollen Hepatocytes Without Inflammation
Needle biopsy of the liver shows diffuse hepatocyte swelling, imparting an overall pale appearance. Steatotic vacuoles are absent or rare. There is no lobular inflammation.

image
Cytologic Features
Liver biopsy from a patient with glycogenic hepatopathy shows diffuse enlargement of hepatocytes with pale, wispy cytoplasm and prominent cytoplasmic membranes image. Glycogenated hepatocyte nuclei image are common.
image
Sparing of Portal Tracts
The portal tracts appear normal in glycogenic hepatopathy image. They contain their normal structures, and there are no portal inflammatory cell infiltrates. The background hepatocytes image are diffusely swollen and filled with glycogen.
image
Hepatocytes: PAS Stain
PAS stain highlights the abundant glycogen in hepatocytes in a liver biopsy from a patient with glycogenic hepatopathy.

TERMINOLOGY

Synonyms

• Hepatic glycogenosis

• Liver glycogenosis
• Glycogen hepatopathy
• Diabetes mellitus-associated glycogen storage hepatomegaly
• Mauriac syndrome
image Liver changes accompanied by growth retardation, delayed puberty, hypercholesterolemia, and cushingoid features

Definitions

• Excessive glycogen storage in hepatocytes secondary to poorly controlled insulin-dependent diabetes mellitus

ETIOLOGY/PATHOGENESIS

Metabolic Factors

• Chronic hyperglycemia due to poorly controlled insulin-dependent diabetes mellitus
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