School of Medicine, Southeast University, Nanjing, China.
National Clinical Research Centre of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China.
Br J Haematol. 2019 Nov;187(4):459-469. doi: 10.1111/bjh.16112. Epub 2019 Jul 26.
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.
肾脏受累在约 70%的系统性免疫球蛋白轻链 (AL) 淀粉样变性患者中被发现。然而,目前尚无针对患者生存的专门用于肾脏 AL 的风险分层系统。半乳糖凝集素-3 (Gal-3) 已被报道在其他肾脏疾病中预示预后不良。我们在 253 例连续诊断为肾脏 AL 的患者的基线样本中测量了 Gal-3 和几种 AL 的传统风险生物标志物。在基线时,Gal-3[风险比 (HR):1.46;P=0.033]、高敏心肌肌钙蛋白 T (hs-cTnT) (HR: 2.65; P<0.001) 和受累与未受累游离轻链之间的差异 (dFLC) (HR: 1.81; P=0.001) 是全因死亡率的独立预测因子。Gal-3、hs-cTnT 和 dFLC 的截断值分别为 20.24ng/ml、0.026ng/ml 和 75.89mg/l。通过为每个截断值以上的三个生物标志物分配 1 分,将患者分为四个阶段。疾病阶段 1、2、3 和 4 的患者比例分别为 17.0%、37.2%、29.2%和 16.6%,中位总生存时间分别为 100、60、29 和 15 个月(P<0.01)。Gal-3 水平越高,死亡风险越大,基于 Gal-3 的风险分层是预测肾脏受累的 AL 淀粉样变性患者死亡风险的可靠模型。