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

BRITISH JOURNAL OF HAEMATOLOGY(2019)

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摘要
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 center dot 46; P = 0 center dot 033], high-sensitivity cardiac troponin T (hs-cTnT) (HR: 2 center dot 65; P < 0 center dot 001) and difference between involved and uninvolved free light chains (dFLC) (HR: 1 center dot 81; P = 0 center dot 001) were independent predictors of all-cause mortality. The cut-off points for Gal-3, hs-cTnT, and dFLC were 20 center dot 24 ng/ml, 0 center dot 026 ng/ml, and 75 center dot 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 center dot 0%, 37 center dot 2%, 29 center dot 2% and 16 center dot 6%, and the median overall survival times from diagnosis were 100, 60, 29 and 15 months, respectively (P < 0 center dot 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.
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关键词
AL amyloidosis,galectin-3,risk stratification,prognosis,survival
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