P-026: A prognostic staging system for light-chain amyloidosis using hepatic and renal indicator data of 1064 Chinese patients

Abstract

<h3>Background</h3> Light-chain (AL) amyloidosis frequently involves severe multiple end-organ damage, thus affecting prognosis. As the current disease staging system is based only on cardiac indicators, herein, we propose a new staging system based on multiple organ indicators to supplement the existing system. <h3>Methods</h3> Patients with AL amyloidosis (n=1064) from 18 Chinese hospitals registered in the nationwide Chinese Registration Network for Light-chain Amyloidosis were enrolled and divided into test and validation cohorts (4:1). Multivariate analyses were performed to identify clinical and laboratory factors for inclusion in the new staging system. A score of 1 was assigned for each of the following—difference between the involved and uninvolved free light chains ≥100 mg/L, estimated glomerular filtration rate <60 mL/min/1.73 m², total bilirubin ≥18 µmol/L, cardiac troponin I ≥0.06 µg/L, and N-terminal pro-brain natriuretic peptide ≥3600 pg/mL—to divide the patients into five disease stages (0 to IV). <h3>Results</h3> Two hundred and twenty (20.7%), 291 (27.3%), 251 (23.6%), 178 (16.7%), and 124 (11.7%) patients had stage 0, I, II, III, and IV disease, respectively. Patients with stages II, III, and IV had median overall survivals of 56.9 months (95% confidence interval [CI], 33.9 to not reached [NR]), 18.6 months (95% CI, 33.9 to NR), and 6.5 months (95% CI, 8.0 to 24.6) (P<0.001), respectively. The 3-year survival estimates for patients with stages 0, I, II, III, and IV were 90.7%, 71.4%, 59.4%, 39.0%, and 22.1%, respectively. <h3>Conclusions</h3> The new prognostic staging system enhances the risk stratification of patients with AL amyloidosis and is useful when multiple organs are involved.