原发性系统性淀粉样变常累及心脏、肾脏和周围神经,肝脏受累的比率可高达97%,但临床多为轻度黄疸,原发性淀粉样变并发重度肝内胆汁瘀积临床少见[1,2],现将以肝内胆汁瘀积为主要表现的肝淀粉样变1例,报告如下。1临床资料患者,男,56岁。因乏力、纳差、尿黄伴右上腹隐痛15d入院,皮 Primary systemic amyloidosis involving the heart, kidney and peripheral nerves, liver involvement rate as high as 97%, but the clinical mostly mild jaundice, primary amyloidosis complicated by severe intrahepatic cholestasis clinical rare [ 1,2], now the liver cholestasis as the main manifestation of liver amyloidosis in 1 case, the report is as follows. 1 clinical data patients, male, 56 years old. Due to fatigue, anorexia, urinary yellow with abdominal pain on the right 15d admission, skin