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Hellenic Archives of Pathology, Volume 19, Issues 1-3, 2005
Ámyloidosis of the liver
Tiniakos D., Thanou A., Kittas Ch.
Laboratory of Histology & Embryology, Medical School,
University of Athens
Amyloidosis is an uncommon metabolic storage disease resulting from deposition of insoluble fibrillar proteins or aberrantly folded protein fragments in a variety of tissues. Although structural conformation of amyloid deposits is similar, precursor proteins vary greatly in derivation. The current classification of amyloidoses, which is essential for therapy and patient prognosis, is based on the type of the precursor protein. The disease can be either systemic or localized. The two major types of systemic amyloidosis are AL (primary or myeloma-associated amyloidosis) and AA (secondary or reactive amyloidosis). In systemic disease the organs most frequently involved are heart, kidneys, gastrointestinal tract, liver and skin. Hereditary types of amyloidosis are also known. Hepatic amyloidosis may be clinically insignificant or may result in liver failure. In AL type liver involvement has no significant impact on prognosis, which is generally poor. The median survival of patients with primary amyloidosis involving the liver is 8,5-9 months. Jaundice or elevated serum bilirubin is a poor prognostic sign. The prognosis is much better in AA than in AL amyloidosis. However, in contrast with the latter, liver involvement in AA amyloidosis is a late manifestation and is associated with a significantly reduced survival rate.
Key words: Liver, amyloidosis, diagnosis, histopathology.
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