Keywords Parole chiave: Amyloidosis, Cholestasis, Hepatomegaly, Portal Hypertension, Alkaline Phosphatase, Aminotransferase, Adult, Aminotransferase Blood Level, Article, Ascites, Case Report, Esophagus Varices, Female, Human, Human Tissue, Priority Journal, Biopsy, Intrahepatic, Fatal Outcome, Liver, Liver Function Tests, Middle Aged,
Affiliations Affiliazioni: Nuclear Medicine Department, University Federico II, Naples, Italy Gastroenterology Unit, University Federico II, Naples, Italy Corso Vittorio Emanuele, 670, 80122 Napoli, Italy
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Primary systemic amyloidosis with giant hepatomegaly and a swiftly progressive course
Although the involvement of the liver is common in systemic amyloidosis (AL), clinical features of hepatic dysfunction and liver chemistry abnormalities are often mild or absent. A mild increase in the serum alkaline phosphatase value is the most common finding. Hypertransaminasemia, hyperbilirubinemia, and portal hypertension with ascites and gastroesophageal varices occur late in the course of the disease and predict a short survival. We describe the case of a 58-year-old woman with AL, whose dramatic and unusual clinical picture, consisting of giant hepatomegaly, hypertransaminasemia, increase in alkaline phosphatase, esophageal varices, and ascites, was rapidly complicated by severe obstructive cholestasis.
Primary systemic amyloidosis with giant hepatomegaly and a swiftly progressive course
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