目的:探讨原发性肝淀粉样变性的临床特点、病理特征、诊断及治疗。方法:报道1例原发性肝淀粉样变性,并复习相关文献。结果:原发性肝淀粉样变性常见临床表现有肝大、ALP及-γGT显著升高,可伴有单克隆蛋白、总胆固醇和血小板升高,病理检查可见淀粉样物质沉积,常用治疗方法为马法兰联合泼尼松。结论:原发性肝淀粉样变性临床罕见,特异性临床表现少,肝穿刺活检为主要确诊手段,治疗以M P方案为主。 Objective: To investigate the clinical features, pathological features, diagnosis and treatment of primary hepatic amyloidosis. Methods: One case of primary hepatic amyloidosis was reported, and the related literature was reviewed. Results: The common clinical manifestations of primary hepatic amyloidosis were hepatomegaly, ALP and -γGT were significantly elevated, may be associated with monoclonal protein, total cholesterol and thrombocytopenia, the pathological examination showed amyloid deposition, the common treatment for Melphalan combined with prednisone. CONCLUSION: Primary hepatic amyloidosis is rare in clinic, with few specific clinical manifestations and liver biopsy as the main diagnostic method. The treatment is based on M P regimen.