A risk stratification for systemic immunoglobulin light‐chain amyloidosis with renal involvement

• Published in: British journal of haematology Vol. 187; no. 4; pp. 459 - 469
• Main Authors: Li, Ting, Huang, Xianghua, Wang, Qingwen, Zhao, Liang, Ren, Guisheng, Chen, Wencui, Zheng, Chunxia, Zhou, Minlin, Jiang, Qi, Yin, Ru, Liu, Zhihong
• Format: Journal Article
• Language: English
• Published: England Blackwell Publishing Ltd 01.11.2019
• Subjects: AL amyloidosis; Amyloidosis; Biomarkers; Biomarkers - analysis; Calcium-binding protein; Galectin 3 - analysis; galectin‐3; Hematology; Humans; Immunoglobulin Light Chains - analysis; Immunoglobulin Light-chain Amyloidosis - complications; Immunoglobulin Light-chain Amyloidosis - diagnosis; Immunoglobulin Light-chain Amyloidosis - mortality; Immunoglobulins; Kidney Diseases - etiology; Light chains; Medical prognosis; Mortality; Predictive Value of Tests; prognosis; Risk Assessment; risk stratification; Survival; Survival Analysis; Troponin; Troponin T; Troponin T - analysis; AL amyloidosis; risk stratification; galectin-3; prognosis; survival
• ISSN: 0007-1048; 1365-2141
• DOI: 10.1111/bjh.16112

Summary Renal involvement is found in about 70% of patients with systemic immunoglobulin light‐chain (AL) amyloidosis. However, there is no risk stratification system specialized for renal AL concerning patients’ survival. Galectin‐3 (Gal‐3) has been reported to portend poor prognosis in other renal diseases. We measured Gal‐3 and several traditional risk biomarkers of AL in baseline samples from 253 consecutive patients diagnosed with renal AL. At baseline, Gal‐3 [Hazard ratio (HR): 1·46; P = 0·033], high‐sensitivity cardiac troponin T (hs‐cTnT) (HR: 2·65; P < 0·001) and difference between involved and uninvolved free light chains (dFLC) (HR: 1·81; P = 0·001) were independent predictors of all‐cause mortality. The cut‐off points for Gal‐3, hs‐cTnT, and dFLC were 20·24 ng/ml, 0·026 ng/ml, and 75·89 mg/l, respectively. Patients were stratified into four stages by assigning a score of 1 for each of the three biomarkers above the cut‐off point. The proportions of patients with disease stages 1, 2, 3 and 4 were 17·0%, 37·2%, 29·2% and 16·6%, and the median overall survival times from diagnosis were 100, 60, 29 and 15 months, respectively (P < 0·01). Higher level of Gal‐3 is associated with increased risk for mortality, and the risk stratification based on Gal‐3 is a reliable model for predicting mortality in AL amyloidosis with renal involvement.