A prognostic staging system for light-chain amyloidosis using hepatic and renal indicator data from 1,064 Chinese patients

Abstract

Background: 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, we propose a new staging system based on multiple organ indicators to supplement the existing system. Methods: Patients with AL amyloidosis (n=1,064) from 18 Chinese hospitals were enrolled and divided into test and validation cohorts (4:1). Multivariate analyses were performed to identify the clinical and laboratory factors for inclusion in the new staging system.Results: A score of 1 was assigned for each of the following-the difference between the involved and uninvolved free light chains ≥100 mg/L, estimated glomerular filtration rate (eGFR) <60 mL/min/1.73 m 2 , total bilirubin (Tbil) ≥18 µmol/L, cardiac troponin I ≥0.06 µg/L, and N-terminal pro-brain natriuretic peptide (NT-proBNP) ≥3,600 pg/mL-to divide the patients into five disease stages (0 to IV). There were 220 (20.7%), 291 (27.3%), 251 (23.6%), 178 (16.7%), and 124 (11.7%) patients with stage 0, I, II, III, and IV disease, respectively. Patients with stage II, III, and IV disease had a median overall survival (OS) of 56.9 months [95% confidence interval (CI), 33.9-not reached (NR)], 18.6 months (95% CI, 33.9-NR), and 6.5 months (95% CI, 8.0-24.6) (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.Conclusions: The new staging system has been developed that incorporates plasma cell-related characteristics in addition to cardiac, renal, and hepatic function parameters. It enhances the risk stratification of patients with AL amyloidosis and is useful when multiple organs are involved.

Chinese collaborative study of survival analysis in 980 patients with AL amyloidosis

“…As a multisystemic disorder, AL amyloidosis can affect the heart, kidneys, liver, nerves, lungs, and bowel [13]. However, the heart and kidney are the two most commonly involved organs [14].…”