Giant hepatomegaly and portal hypertension in an elderly patient with primary liver amyloidosis: an uncommon clinical occurrence.

Primary systemic amyloidosis (AL) is an uncommon disease characterized by the extracellular deposition of a protein with a beta-fibrillar structure, consisting of monoclonal immunoglobulin light chains, λ or κ (ratio of λ to κ, 3:1). In systemic amyloidosis liver involvement is frequent but it rarely has clinical importance. The massive and localized liver deposition of amyloid, characterized by marked hepatomegaly and portal hypertension without hepato-cellular failure and by a severe prognosis, without sys- temic involvement, is less frequent. The authors describe an unusual case of pri- mary hepatic amyloidosis with giant he- patomegaly, intrahepatic cholestasis, portal hy- pertension and splenomegaly, occurred in an el- derly patient.