From What’s Your Diagnosis? on page 9
Liver biopsy demonstrated diffuse clearing of cy- toplasm of the hepatocytes with strong periodic acid–Schiff positivity. There was no evidence of ibrosis, steatosis, inflammation, iron staining, or Mallory bodies. Combined with the clinical history of poorly controlled type 1 diabetes, these liver biopsy findings were consistent with glycogenic hepatopathy.
Although the patient had alpha-1-antitrypsin deficiency,
it was not believed to have contributed to his elevated
liver enzymes because there were minimal scattered al-
pha-1-antitrypsin globules and no inflammation or fibrosis
on biopsy. With better glycemic control, the patient’s liver
enzymes returned to normal levels. Unfortunately, the
patient had a relapse because his diabetes again became
Glycogenic hepatopathy is a disease process in which
abnormal glycogen deposits in the liver, causing elevation
of serum transaminases.1 It is usually seen in patients with
poorly controlled type 1 diabetes, with clinical signs and
symptoms including abdominal pain, nausea, vomiting, and
hepatomegaly. The key histologic findings of glycogenic
hepatopathy are swollen and pale-staining hepatocytes on
hematoxylin and eosin stains and extensive glycogen accu-
mulation seen on periodic acid–Schiff stains. Other histo-
logic features include prominent glycogenated nuclei, giant
mitochondria, and scattered acidophilic bodies. 2 The marked
accumulation of glycogen in hepatocytes is believed to cause
hepatomegaly and leakage of transaminases. 3 Rapid enlarge-
ment of the liver results in stretching of the liver capsule
and abdominal pain. n
1. Torbenson, M., et al. Glycogenic hepatopathy: an underrecognized hepatic
complication of diabetes mellitus. Am J Surg Pathol. 2006;30:508-13.
2. Sweetser, S., Kraichely, R. The bright liver of glycogenic hepatopathy. Hepatology. 2010;51:711-2.
3. Fridell, J., S., et al. Complete reversal of glycogen hepatopathy with pancreas transplantation: two cases. Transplantation. 2007;83:84-6.