Objective: To analyze the prognostic value of baseline serum free light chain (sFLC) in immunoglobulin light-chain cardiac amyloidosis (AL-CA) . Methods: Thirty patients diagnosed with AL-CA from January 2012 to December 2016 at Beijing Chaoyang Hospital were included in this study to retrospectively evaluate the clinical data. The cut-off value of dFLC (involved sFLC minus uninvolved sFLC) was determined according to the receiver operator characteristic curve (ROC) and grouped, the prognoses of both groups were evaluated. Results: The onset age of all AL-CA patients was 57 years old. It occurred more commonly in men (21 cases, 70%) and the light chains of immunoglobulin composed mainly of type λ (22 cases, 73.3%) . Renal involvements occurred in 17 cases (56.7%) . The median value of difference between involved and uninvolved serum immunoglobulin free light chain levels (dFLC) was 162.9 (57.9-401.6) mg/L. More subjects in the high dFLC group had higher BNP (P=0.005) , and shorter median survival than those in the low dFLC group (15 months vs 47 months, P<0.001) . Similar results of median survival were observed when the patients were redivided by a new cut-off value of 180 mg/L for dFLC (high dFLC group: 22 months, low dFLC group: 40 months, P=0.001) , or a κ/λ ratio in which patients with κ type sFLC-ratio<3.79 and λ type sFLC-ratio≥0.06 were grouped into the low sFLC-ratio (37 months) , and the reverse the high sFLC-ratio ones (25 months, P=0.021) . In multivariate analysis, dFLC and New York Heart Association (NYHA) classification of cardiac function were two risk factors associated with all-cause mortality in patients, of them the hazard ratio for higher dFLC was 12.13 (95%CI 2.98-49.30, P<0.001) . Conclusion: Measurement of the sFLC level could implicate the prognosis of AL-CA.
目的： 探讨血清游离轻链（sFLC）检测在免疫球蛋白轻链型心肌淀粉样变性（AL-CA）患者中的预后价值。 方法： 回顾性分析2012年1月至2016年12月于北京朝阳医院确诊为AL-CA且有完整sFLC数据的30例患者，通过受试者工作特征（ROC）曲线及Mayo2012分期确定受累轻链与非受累轻链差值（dFLC）的界点并分组评估患者预后。 结果： 30例AL-CA患者中位年龄57岁，男女比例21∶9。λ型淀粉样变22例（73.3%），累及肾脏者17例（56.7%）。30例AL-CA患者的中位dFLC为162.9（57.9~401.6）mg/L，高dFLC组的B型利钠肽（BNP）水平较低dFLC组高（3 966 ng/L对2 142 ng/L，P=0.005），中位生存时间缩短（15个月对47个月，P<0.001）。以Mayo2012分期所规定的dFLC=180 mg/L为界值将患者重新分组并进行Kaplan-Meier生存分析，高dFLC组较低dFLC组的中位生存时间仍缩短（22个月对40个月，P=0.001）。以κ型和λ型患者的κ/λ轻链比值中位数为界值，将κ型sFLC比值<3.79和λ型sFLC比值≥0.06归为低sFLC比值组，反之为高sFLC比值组，两组患者的生存分析显示，高sFLC比值组较低sFLC比值组中位生存时间缩短（25个月对37个月，P=0.021）。Cox比例风险模型进行多因素分析显示，dFLC和美国纽约心脏病学会心功能分级是影响患者生存时间的因素，其中高dFLC（dFLC>162.9 mg/L）组的死亡风险是低dFLC（dFLC≤162.9 mg/L）组的12.13（95% CI 2.98~49.30，P<0.001）倍。 结论： sFLC检测能够有效地提示AL-CA患者的预后。.
Keywords: Amyloidosis; Myoeardium; Prognosis; Serum free light chain.