Decrease of B-type natriuretic peptide to less than 200 pg/mL predicts longer survival in cardiac immunoglobulin light chain amyloidosis
Decrease of B-type natriuretic peptide to less than 200 pg/mL predicts longer survival in cardiac immunoglobulin light chain amyloidosis
复制标题
DOI：
10.1007/s12185-015-1814-0
复制
发表时间：
2015-08-01
影响因子：
2.1
通讯作者：
Shinomura, Yasuhisa
中科院分区：
文献类型：
--
作者：
Ishiguro, Kazuya;Hayashi, Toshiaki;Shinomura, Yasuhisa
免疫球蛋白轻链（AL）淀粉样变性是由异常浆细胞产生的单克隆轻链片段组成的不溶性淀粉样纤维沉积引起的全身性疾病。据报道，预后差;中位生存时间（MST）总体为1-2年，心脏受累患者为6个月。我们在此报告了2008年1月至2012年12月在我院治疗的24例AL淀粉样变性患者的治疗结果，其中包括11例心脏受累患者。在心脏受累患者中，从诊断开始的MST明显较短（9.8个月）。其中，治疗后B型利钠肽（BNP）降低至< 200 pg/mL的患者比未降低的患者生存时间更长（MST：未达到vs. 6.1个月; p = 0.003，对数秩检验）。BNP降至< 200 pg/mL的中位时间为6.3个月。治疗期间BNP下降至200 pg/mL或更低预示心脏AL淀粉样变性患者的生存期更长。
Immunoglobulin light chain (AL) amyloidosis is a systemic disorder caused by depositions of insoluble amyloid fibrils that are composed of fragments of monoclonal light chains produced by abnormal plasma cells. The prognosis is reported to be poor; median survival time (MST) is 1-2 years overall, and is 6 months in patients with cardiac involvement. We here report the treatment outcomes of 24 patients with AL amyloidosis at our hospital between January 2008 and December 2012, including 11 patients with cardiac involvement. MST from the diagnosis was significantly shorter (9.8 months) in patients with cardiac involvement. Of these, patients who achieved a decrease of B-type natriuretic peptide (BNP) to < 200 pg/mL after treatment survived longer than patients who did not (MST: not reached vs. 6.1 months; p = 0.003, log-rank test). The median time to decrease BNP to < 200 pg/mL was 6.3 months. The decline of BNP to 200 pg/mL or less during treatment predicts longer survival in patients with cardiac AL amyloidosis.