血清游离轻链检测在轻链型心肌淀粉样变性中的预后价值  

The prognostic value of baseline serum free light chain in immunoglobulin light-chain cardiac amyloidosis

作　　者：王立明[1] 王桐桐 田颖[2] 赵蕾 杨新春 陈文明[2] Wang Liming;Wang Tongtong;Tian Ying;Zhao Lei;Yang Xinchun;Chen Wenming(Department of Geriatrics,Beijing NO.6 Hospital,Beijing 100007,China;Department of Hematology,Beijing Chaoyang Hospital of Capital Medical University,Beijing 100020,China;Department of Cardiology,Beijing Chaoyang Hospital of Capital Medical University,Beijing 100020,China)

机构地区：[1]北京市第六医院老年干部科,100007 [2]首都医科大学附属北京朝阳医院血液内科,100020 [3]首都医科大学附属北京朝阳医院心脏中心,100020

出　　处：《中华血液学杂志》2020年第1期47-53,共7页Chinese Journal of Hematology

基　　金：中国博士后基金(2018M641419);北京市博士后工作经费资助项目(2018-22-114)。

摘　　要：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(3966 ng/L对2142 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。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 typeλ(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 aκ/λratio in which patients withκtype sFLC-ratio<3.79 andλtype 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.

关 键 词：血清游离轻链 淀粉样变性 心肌 预后 

分 类 号：R542[医药卫生—心血管疾病]