Talk:Medical liver disease

TPN

Microscopic description

The specimen consists of liver cores with at least twelve partial portal tracts. There is a moderate macrovesicular steatosis without clear zonation. There are no mallory bodies.

There is marked cholestasis and focal hepatocyte enlargement with nuclear pyknosis and karyolysis (feathery degeneration). There is no interface inflammation or lobular inflammation and only mild portal inflammation, that is predominantly mononuclear. There is a suggestion of scant, mild iron deposition at high power and mild-to-moderate periportal copper deposition. There is no obvious bile duct injury on routine stains. Cytokeratin staining (panker) demonstrates bile ductular proliferation. There is marked fibrotic portal expansion, with thick fibrous septa. There are rare rounded septa, suggestive of focal nodule formation; Laennec fibrosis stage is II-III / IV. The PAS and PAS-D stains are non-contributory.

Final diagnosis

Liver, core biopsy -
i) Macrovesicular steatosis, moderate.
ii) Cholestasis with bile ductule proliferation.
iii) Moderate-to-marked fibrosis, periportal; Laennec fibrosis stage II-III / IV.

See diagnosis comment.

Comment

The histomorphologic findings (cholestasis, fibrosis and steatosis) are compatible changes seen in total parenteral nutrition (TPN).

Glycogen storage disease

Microscopic description

The sections show hepatocytes with moderate enlargement and pale cytoplasm diffusely, with cobweb-like cytoplasmic material. Focally, hepatocytes have marked enlargement with pynotic nuclei. There are ten portal tracts present in the specimen.

There is minimal focal portal inflammation and moderate inflammation of the lobule, characterized by eosinophils and neutrophils, with focal hepatocyte necrosis. There is no inflammation at the interface. The Laennec inflammation grade is I-II/IV. There is significant fibrosis, which includes the presence of portal expansion and septa; the Laennec fibrosis stage is II / IV.

There is no cholestasis, no iron deposition, no bile duct injury and no copper deposition. PAS staining is equivocal for glycogen deposition. PAS-D marks scattered, predominantly periportal, macrophages.

Electron microscopy demonstrates electron dense material consistent with glycogen that is not membrane bound.

Final diagnosis

Liver, core biopsy -
i) Changes consistent with glycogen storage disease, see comment.
ii) Fibrotic portal expansion and focal septa (Laennec fibrosis stage II / IV).

Comment

The periportal predominant fibrosis, diffuse hepatocyte distension with whispy and pale cytoplasm, and non-membrane bound electron dense (glycogen-like) material, are suggestive of glycogen storage disease type III (Cori disease).