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 t he variable infiltration of multiple organs by a fibrillar protein cal led 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.