关键词: 肝脏淀粉样变性, 轻链型淀粉样变性, 临床表现, 预后

Abstract:

Ｏｂｊｅｃｔｉｖｅ：To evaluate clinical manifestation and prognosis of hepatic systemic light chain (AL) amyloidosis.
Ｍｅｔｈｏｄｏｌｏｇｙ：We retrospectively analyzed the clinical data and outcomes of 81 patients diagnosed with hepatic AL amyloidosis.
Ｒｅｓｕｌｔｓ：The median age was 55,with a male to female ratio of 29∶1. The main clinical manifestations included edema,digestive symptoms,weight loss,fatigue and ascites.Among 51 patients who received treatment,the therapeutic effect could be evaluated in 34 patients. 19 patients (559%) achieved hematological remission,and the risk of death in patients who did not achieve hematological remission was 66 times higher than that in patients achieved hematological remission. The median survival time was 9 months,with a 1,3 and 5year survival rate of 310%,178% and 107%,respectively. Multivariate analysis showed that baseline NTproBNP≥1 800 pg/ml was an independent risk factor for prognosis.
Ｃｏｎｃｌｕｓｉｏｎ：The clinical manifestations of hepatic AL amyloidosis are diverse and the prognosis is poor. The significantly elevated NTproBNP indicates poor outcome. Early diagnosis and timely treatment may improve the prognosis.

Key words: hepatic amyloidosis, light chain amyloidosis, clinical manifestationprognosis