PRIMARY SYSTEMIC AMYLOIDOSIS WITH GIANT HEPATOMEGALY AND A SWIFTLY PROGRESSIVE COURSE

Although the involvement of the liver is common in systemic amyloidosi s (AL), clinical features of hepatic dysfunction and liver chemistry a bnormalities are often mild or absent. A mild increase in the serum al kaline phosphatase value is the most common finding. Hypertransaminase mia, hyperbilirubinemia, and portal hypertension with ascites and gast roesophageal varices occur late in the course of the disease and predi ct a short survival. We describe the case of a 58-year-old woman with AL, whose dramatic and unusual clinical picture, consisting of giant h epatomegaly, hypertransaminasemia, increase in alkaline phosphatase, e sophageal varices, and ascites, was rapidly complicated by severe obst ructive cholestasis.