Glycogenic Hepatopathy

Published on 21/04/2017 by admin

Filed under Pathology

Last modified 22/04/2025

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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
• Longstanding high blood sugar levels lead to glycogen accumulation in hepatocytes
• Hepatomegaly and transaminase elevations attributed to this excess glycogen accumulation

CLINICAL ISSUES

Epidemiology

• Age

image Occurs in both children and adults

Presentation

• Abdominal pain
• Hepatomegaly
• Nausea
• Vomiting
• History of ketoacidosis

Laboratory Tests

• Hyperglycemia
• Elevated transaminases
• Alkaline phosphatase may also be elevated
• Elevated hemoglobin A1c (HbA1c) indicates history of poor glycemic control

Treatment

• Options, risks, complications

image Mainstay treatment is improved management of diabetes mellitus
– Optimization of glycemic control with insulin and diet
• Surgical approaches

image Resolution after pancreas transplantation has been reported

Prognosis

• Excellent outcome with medical management
• Liver histology improves and transaminases normalize with optimization of glycemic control

IMAGING

Radiographic Findings

• Hyperdense liver on CT scan without administration of contrast material

MACROSCOPIC

General Features

• Hepatomegaly

MICROSCOPIC

Histologic Features

• Diffuse pale-staining hepatocyte cytoplasm
• PAS stain confirms excessive glycogen accumulation in hepatocytes
• Rare acidophil bodies may be seen
• Glycogenated hepatocyte nuclei
• Notable absence of inflammation or other features of hepatic injury
• Fibrosis is rare

ANCILLARY TESTS

Electron Microscopy

• Marked glycogen accumulation in hepatocyte cytoplasm and nuclei

DIFFERENTIAL DIAGNOSIS

Normal Liver

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

Fatty Liver Disease

• Pale, enlarged hepatocytes may be misinterpreted as ballooning hepatocyte degeneration
• Most cases of glycogenic hepatopathy show little or no steatosis

Glycogen Storage Disease

• Typically presents at younger age and without history of diabetes mellitus

DIAGNOSTIC CHECKLIST

Clinically Relevant Pathologic Features

• Marked transaminase elevation attributed to excessive glycogen accumulation in hepatocytes

Pathologic Interpretation Pearls

• Consider in patients with poorly controlled diabetes mellitus and unexplained hepatomegaly &/or transaminase elevations

• Pale, slightly swollen hepatocytes with prominent cell membranes
image May be mistaken for normal hepatocytes, glycogen storage disease, or fixation artifact

SELECTED REFERENCES

1.Sweetser, S, et al. The bright liver of glycogenic hepatopathy. Hepatology. 2010; 51(2):711–712.

2.Fridell, JA, et al. Complete reversal of glycogen hepatopathy with pancreas transplantation: two cases. Transplantation. 2007; 83(1):84–86.

3.Torbenson, M, et al. Glycogenic hepatopathy: an underrecognized hepatic complication of diabetes mellitus. Am J Surg Pathol. 2006; 30(4):508–513.

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