中国实用内科杂志2009，Vol.29Issue(11)：1003-1005,3.
心脏原发轻链型淀粉样变临床特点和生存分析
Clinical characteristics and survival in patients with heart involvement in primary immunoglobulin light-chain amyloidosis (AL)
摘要
Abstract
Objective To study clinical features and analyze survival of patients with AL-type cardiac amyloidosis, nethotis Between January 1998 and March 2008,25 patients with primary AL amyloidosis were evaluated and followed up. Results Of the 25 patients with AL, ten were identified with cardiac amyloidosis. Median age at presentation was 55 years (range 38～62). All but one patients had extracardiac involvement or mostly kidney. Dyspnea, weakness and fatigue were the common presenting symptoms. Seven of ten patients had congestive heart failure (CHF) with predominantly right-sides failure signs. Echocardiogram revealed concentric hypertrophy of the left and right ventricles. With a median 6.8-month (range,0.1 to 76.7-month) follow-up from diagnosis,the estimated median survival (MS) of all 25 patients was 16.4 months. 14 patients died and 12 died of heart and renal failures. MS of 10 patients with cardiac AL was 1.4 months, significantly shorter compared to patients without cardiac amyioid (30.2 months, P = 0.006). Conclusion AL cardiac amyloidosis was frequently associated with multiorgan involvement. The characteristic clinical feature was CHF. Heart involvement in AL amyloidosis,particularly with the onset of heart failure, represented the worst prognostic indica- tor. Heart failure was the main cause of death in AL patients.关键词
轻链型淀粉样变/心脏/生存分析Key words
immunoglobulin light-chain amyloidosis/ survival分类
医药卫生引用本文复制引用
王荷花,颜绵生,许多荣,童秀珍,陈文芳,李娟..心脏原发轻链型淀粉样变临床特点和生存分析[J].中国实用内科杂志,2009,29(11):1003-1005,3.基金项目
广东省自然博士启动基金(07300956) （07300956）
