Chinese Journal of Nephrology, Dialysis & Transplantation ›› 2019, Vol. 28 ›› Issue (4): 318-323.DOI: 10.3969/j.issn.1006-298X.2019.04.004
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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 29∶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 (559%) achieved hematological remission,and the risk of death in patients who did not achieve hematological remission was 66 times higher than that in patients achieved hematological remission. The median survival time was 9 months,with a 1,3 and 5year survival rate of 310%,178% and 107%,respectively. Multivariate analysis showed that baseline NTproBNP≥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 NTproBNP indicates poor outcome. Early diagnosis and timely treatment may improve the prognosis.
Key words: hepatic amyloidosis, light chain amyloidosis, clinical manifestationprognosis
ZHAO Liang,REN Guisheng,GUO Jinzhou, et al. Clinical manifestation and prognosis of liver systemic light chain amyloidosis[J]. Chinese Journal of Nephrology, Dialysis & Transplantation, 2019, 28(4): 318-323.
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URL: http://www.njcndt.com/EN/10.3969/j.issn.1006-298X.2019.04.004
http://www.njcndt.com/EN/Y2019/V28/I4/318