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伴有肾脏受累的系统性免疫球蛋白轻链淀粉样变性的风险分层。

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

机构信息

School of Medicine, Southeast University, Nanjing, China.

National Clinical Research Centre of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China.

出版信息

Br J Haematol. 2019 Nov;187(4):459-469. doi: 10.1111/bjh.16112. Epub 2019 Jul 26.

Abstract

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.

摘要

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。

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