Wang L M, Wang T T, Tian Y, Zhao L, Yang X C, Chen W M
Department of Geriatrics, Beijing NO.6 Hospital, Beijing 100007, China.
Department of Hematology, Beijing Chaoyang Hospital of Capital Medical University, Beijing 100020, China.
Zhonghua Xue Ye Xue Za Zhi. 2020 Jan 14;41(1):47-53. doi: 10.3760/cma.j.issn.0253-2727.2020.01.009.
To analyze the prognostic value of baseline serum free light chain (sFLC) in immunoglobulin light-chain cardiac amyloidosis (AL-CA) . 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. 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 (=0.005) , and shorter median survival than those in the low dFLC group (15 months 47 months, <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, =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, =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% 2.98-49.30, <0.001) . Measurement of the sFLC level could implicate the prognosis of AL-CA.
分析基线血清游离轻链（sFLC）在免疫球蛋白轻链型心脏淀粉样变性（AL-CA）中的预后价值。本研究纳入了2012年1月至2016年12月在北京朝阳医院确诊为AL-CA的30例患者，对其临床资料进行回顾性评估。根据受试者工作特征曲线（ROC）确定dFLC（受累sFLC减去未受累sFLC）的截断值并进行分组，评估两组的预后。所有AL-CA患者的发病年龄为57岁。男性更为常见（21例，70%），免疫球蛋白轻链主要由λ型组成（22例，73.3%）。17例（56.7%）出现肾脏受累。受累与未受累血清免疫球蛋白游离轻链水平差异（dFLC）的中位数为162.9（57.9 - 401.6）mg/L。高dFLC组中更多受试者的脑钠肽（BNP）更高（P = 0.005），且中位生存期比低dFLC组短（15个月对47个月，P < 0.001）。当以dFLC新的截断值180 mg/L重新分组患者时，观察到类似的中位生存期结果（高dFLC组：22个月，低dFLC组：40个月，P = 0.001），或者以κ/λ比值分组，κ型sFLC比值<3.79且λ型sFLC比值≥0.06的患者分为低sFLC比值组（37个月），反之则为高sFLC比值组（25个月，P = 0.021）。在多因素分析中，dFLC和纽约心脏协会（NYHA）心功能分级是与患者全因死亡率相关的两个危险因素其中dFLC升高的风险比为12.13（95% 2.98 - 49.30，P < 0.001）。sFLC水平的检测可提示AL-CA的预后。
