Primary systemic amyloidosis with giant hepatomegaly and portal hypertension: a case report and a review of the literature.
Amyloidosis is a manifestation of a group of diseases resulting from the variable infiltration of multiple organs by a fibrillar protein called amyloid. Hepatic involvement in amyloidosis is common both in the primary and in the secondary forms, whereas clinically-dominant liver amyloidosis is relatively rare. The authors describe a case of primary systemic amyloidosis with giant hepatomegaly, portal hypertension and renal insufficiency; the patient did not develop jaundice, ascites or gastrointestinal bleeding but died 6 months later, death being due to cerebral haemorrhage.
Serra
L
L
Istituto di Clinica Medica, Università di Modena, Italy.
Poppi
M C
MC
Criscuolo
M
M
Zandomeneghi
R
R
D000328:Adult; D000686:Amyloidosis / Q000150:complications / Q000473:pathology*; D001706:Biopsy; D005753:Gastric Mucosa / Q000473:pathology; D006529:Hepatomegaly / Q000209:etiology / Q000473:pathology*; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000473:pathology*; D008099:Liver / Q000473:pathology; D008297:Male