title: Hepatomegaly, weight loss and general malaise - the first manifestations of primary systemic amyloidosis.
abstract: Amyloidosis is characterised by the accumulation of poorly soluble fibrous proteins in the extracellular space of various bodily organs. Light chain amyloidosis (AL) is recognised as the most common form of systemic amyloidosis. Light chains are deposited in the majority of bodily organs, and accumulation of them in the liver produces hepatomegaly. We report a case of AL-systemic amyloidosis with liver involvement in a 71-year-old woman. Hepatomegaly, weight loss and general malaise were the first manifestations of the disease. Liver biopsy found amyloid deposits along the sinusoids as well as in the space of Disse, inside the vascular wall and in connective tissue of the portal tracts, which showed a positive reaction in Congo Red stain. Further diagnosis showed the presence of systemic amyloidosis. The patient was put on cyclophosphamide and steroid therapy.
authors.affiliation: Department of Gastroenterology, Medical University of Lublin, Poland.
authors.forename: Halina
authors.initials: H
authors.lastname: Cichoż-Lach
authors.affiliation: Department of Gastroenterology, Medical University of Lublin, Poland.
authors.forename: Beata
authors.initials: B
authors.lastname: Prozorow-Król
authors.affiliation: Department of Clinical Pathomorphology, Medical University of Lublin, Poland.
authors.forename: Jarosław
authors.initials: J
authors.lastname: Swatek
authors.affiliation: Department of Gastroenterology, Medical University of Lublin, Poland.
authors.forename: Barbara
authors.initials: B
authors.lastname: Skrzydło-Radomańska
authors.affiliation: 1 Department of Radiology, Medical University of Lublin, Poland.
authors.forename: Leszek
authors.initials: L
authors.lastname: Buk
authors.affiliation: Department of Clinical Pathomorphology, Medical University of Lublin, Poland.
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Zdunek
authors.affiliation: Department of Gastroenterology, Medical University of Lublin, Poland.
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Kowalik
authors.affiliation: Department of Gastroenterology, Medical University of Lublin, Poland.
authors.forename: Maria
authors.initials: M
authors.lastname: Słomka
keywords: amyloidosis; hepatomegaly; histological diagnosis