Efficacy of Chemotherapy for Light-Chain Amyloidosis in Patients Presenting With Symptomatic Heart Failure
Efficacy of Chemotherapy for Light-Chain Amyloidosis in Patients Presenting With Symptomatic Heart Failure
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DOI：
10.1016/j.jacc.2016.03.593
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发表时间：
2016-06-28
影响因子：
24
通讯作者：
Hanna, Mazen
中科院分区：
文献类型：
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作者：
Sperry, Brett W.;Ikram, Asad;Hanna, Mazen
背景 伴有心脏受累的轻链淀粉样变性（AL）预后不良；对 AL 淀粉样变性引起的心力衰竭进行了回顾性研究。裁定初始治疗策略，并使用倾向评分分析来调整治疗的非随机分配。在调整接受治疗的倾向评分、年龄、NYHA 功能分级和射血分数后，使用 Cox 比例风险模型评估生存率。 结果 在 106 名接受治疗的患者（年龄 64.6 +/- 11.3 岁，63% 男性，76% lambda 亚型）中，40 名接受 3 种药物治疗方案，66 名接受其他治疗方案。总体死亡率为 65%，BDex+AA 队列的死亡率为 48%（中位生存时间 821 天），接受其他治疗方案的患者死亡率为 76%（中位生存时间 223 天）。 BDex+AA 初始治疗与多变量调整后死亡率降低相关（风险比：0.209；95% 置信区间：0.069 至 0.636；p = 0.006）。在进一步调整 Mayo 分期的组成部分后，这种相关性仍然存在。结论：使用 BDex+AA 治疗有症状心力衰竭患者的 AL 淀粉样变性与调整临床变量后生存率的提高相关。 (C) 2016 年，美国心脏病学会基金会。
BACKGROUND Light-chain amyloidosis (AL) with cardiac involvement carries a poor prognosis; median untreated survival is = II heart failure due to AL amyloidosis were retrospectively studied. Initial treatment strategy was adjudicated and propensity score analysis was used to adjust for the nonrandomized allocation of treatments. Survival was assessed using a Cox proportional hazards model after adjusting for the propensity score for receiving treatment, age, NYHA functional class, and ejection fraction.RESULTS Among 106 treated patients (age 64.6 +/- 11.3 years, 63% male, 76% lambda subtype), 40 received the 3-drug regimen and 66 received other regimens. Mortality was 65% overall, 48% in the BDex+AA cohort (median survival time 821 days), and 76% in patients who received other regimens (median survival time 223 days). Initial treatment with BDex+AA was associated with decreased mortality after multivariable adjustment (hazard ratio: 0.209; 95% confidence interval: 0.069 to 0.636; p = 0.006). This association remained after further adjustment for components of the Mayo Stage.CONCLUSIONS Use of BDex+AA in the treatment of AL amyloidosis in patients presenting with symptomatic heart failure is associated with improved survival after adjusting for clinical variables. (C) 2016 by the American College of Cardiology Foundation.