Amyloidose der Leber

A case of
hepatic amyloidosis with CT, MRI and angiographic findings. Homogeneous hypoattenuation of liver and mild hepatosplenomegaly.
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A case of
hepatic amyloidosis with CT, MRI and angiographic findings. Mild hepatosplenomegaly and diffuse increase of T1 signal in the liver parenchyma on T1 fat saturation image.
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A case of
hepatic amyloidosis with CT, MRI and angiographic findings. Marked narrowing of hepatic arteries
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A case of
hepatic amyloidosis with CT, MRI and angiographic findings. Liver demonstrated heterogeneous enhancement with patchy and geographic pattern.
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A case of
hepatic amyloidosis with CT, MRI and angiographic findings. Periportal hypoenhancement in bilateral hepatic lobes.
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A case of
hepatic amyloidosis with CT, MRI and angiographic findings. Diffuse stenosis of the coeliac trunk, common hepatic artery and intrahepatic arteries, with intraluminal irregularities.
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Hepatic amyloidosis refers to the extracellular deposition of amyloid in the parenchyma sinusoids or vessel walls. Liver involvement in amyloidosis is uncommon.

Pathology

It can be primary or secondary and it typically occurs as diffuse infiltration .

There is amyloid deposition in liver parenchyma which occurs along the sinusoids within the space of Disse, or in blood vessel walls. Hepatocytes can be markedly compressed by extensive accumulation of amyloid and they may atrophy or nearly disappear.

In advanced cases with massive infiltration, the liver is enlarged with a rubbery elastic consistency, and it may show 'lardaceous liver' appearance.

Radiographic features

Hepatomegaly and heterogeneous appearances of the liver parenchyma are the most common, though nonspecific, imaging features .

Ultrasound

Non-specific and may show heterogeneous hepatic echotexture or diffuse or focal areas of low attenuation . Prominent calcifications can be rarely seen.

CT

There can be hepatomegaly with decreased hepatic attenuation . Some calcification may be present.

MRI 
  • T1: has increased signal through the liver
  • T2: little change

Differential diagnosis

For CT appearances of decreased hepatic attenuation: see causes of decreased hepatic attenuation.

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu Amyloidose der Leber: