胃腸および消化器系

抽象的な

Liver Amyloidosis Complicated with Liver Failure: A Case Report and Review of the Literature

Mona AL-Saedi, Faisal Batwa, Yaser Dahlan, Abdul Halim Kinsara, Ahmed Absi and Mohamed Satti

Introduction: Liver involvement by amyloidosis is not uncommon. The clinical presentation is usually subclinical or with mild elevation of liver enzymes. Liver failure and rupture has rarely been reported. We report a case that presented with new onset ascites and rapidly progressed into liver failure and rupture with fatal outcome. Methods: A 53 years old male patient presented with progressive anasarca over 2 months. His evaluation revealed ascites with high serum-ascites albumin gradient. Liver imaging suggested mild hepatomegaly with nodular liver and splenomegaly with patent hepatic and portal veins. Cardiac workup was unremarkable. Non nephrotic range proteinuria was noted with hypoalbuminemia and mild hyperbilirubinemia. Esophageal varices were seen on gastroscopy. After thorough blood tests, the patient was labelled as cryptogenic cirrhosis and started on diuretics. Result: One week later he developed transient renal failure and confusional state. His serum bilirubin started to increase rapidly .Under evaluation for liver transplant, the patient underwent liver biopsy which showed massive deposits of amyloid with atrophy of hepatocytes. Rectal biopsy was unremarkable. Bone marrow biopsy showed hypercellular marrow with 5-10% abnormal looking plasma cells some appeared in abnormal clusters with thickened blood vessels which were positive for congo red stain. Kappa and lambda immunochemical stains were inconclusive and cytogenetic studies were not performed. Serum protein electrophoresis was normal. Urinary Bence-Jones proteins were negative. Skeletal survey could not be done due to the patient’s critical condition. Five days later the patient developed hypotension with leukocytosis and was transferred to the Intensive Care Unit with the impression of septic shock. Ascetic tap showed frank blood and computed tomography confirmed rupture of the liver. His liver enzymes and bilirubin continued to deteriorate and he developed refractory coagulopathy. A trial of hepatic artery embolization was unsuccessful and the patient succumbed shortly after. Conclusion: Liver amyloidosis can be fatal. It should be considered in any patient who present with features of ascites and hepatomegaly.