1.
Arab Journal of Gastroenterology. 2010; 11 (3): 167-170
in English
| IMEMR
| ID: emr-145072
ABSTRACT
Liver involvement in systemic amyloidosis is not uncommon; however, presentation with jaundice is rare. It is considered a preterminal sign. We present four cases of primary AL amyloidosis with severe intrahepatic cholestasis, two of whom presenting also with features of portal hypertension with oesophageal varices. Histopathology revealed diffuse hepatic amyloid deposits in the lobules, portal tracts and sinusoidal space. Review of literature reveals that hepatic amyloidosis presenting with cholestasis and portal hypertension is unusual and portends a poor prognosis