%0 Journal Article
%T Fatal Subacute Hepatic Failure in a Patient with AA-Type Amyloidosis: Case Report
%A Ibrahim Altraif
%A Fayaz A. Handoo
%A Khaled O. Alsaad
%A Adel Gublan
%J Pathology Research International
%D 2010
%I Hindawi Publishing Corporation
%R 10.4061/2010/648089
%X Although systemic amyloidosis of amyloid-associated protein (AA) type (secondary or reactive amyloidosis) frequently involves the liver, it rarely causes clinically apparent liver disease. Mild elevation of alkaline phosphatase and hepatomegaly are the most common biochemical and clinical findings, respectively. We report a case of systemic amyloidosis of AA type, which clinically presented as subacute hepatic failure and resulted in a fatal clinical course in a 69-year-old man. To the best of our knowledge, this is the fifth case of hepatic amyloidosis of AA type that clinically presented as fatal subacute hepatic failure, an unusual clinical presentation for hepatic involvement by systemic AA-type amyloid. 1. Introduction Hepatic involvement in systemic amyloidosis is common and occurs in myeloma-related (AL) (primary amyloidosis) and amyloid-associated (AA) (secondary or reactive) types of the disease. However significant clinical evidence of hepatic dysfunction is not common and the patients rarely exhibit clinical manifestations. These may include hepatomegaly, mild jaundice, and, rarely, severe cholestasis. Portal hypertension may complicate hepatic amyloidosis, and subcapsular hematoma and spontaneous rupture of liver were reported [1]. Subacute and acute, fulminant liver failures were previously reported, particularly in association with AL-type amyloidosis in the setting of myeloma [2]. Herein, we present an unusual case of AA-type amyloidosis with massive hepatic involvement and subacute liver failure〞a clinical presentation rarely seen in secondary hepatic amyloidosis. 2. Case Report A previously healthy 69-year-old man presented with history of progressively increasing jaundice for six weeks and abdominal distension with swelling of the feet for four weeks. He also had symptoms of fatigue, poor appetite, and mild generalized itching. His past medical history was unremarkable, with no previous history of chronic liver disease. Upon physical examination, the patient had normal built exhibited severe jaundice, bilateral pitting lower-limb edema, left-sided moderate pleural effusion, short apical systolic murmur, and massive hepatomegaly, associated also with marked ascites without signs of overt congestive heart failure. Complete blood count and serum electrolytes were within normal limits. Laboratory studies showed blood urea 3.9ˋmmol/l (normal range 3每9.2), creatinine 64 mol/l (45每110), total bilirubin of 196ˋ mol/l (3.4每20.5), serum albumin 24ˋg/l (35每50), INR 3.2, alkaline phosphatase 256 IU/l (40每150), 污-glutamyltransferase 136 (2每30),
%U http://www.hindawi.com/journals/pri/2010/648089/