Clinical characteristics and survival in patients with heart involvement in primary immunoglobulin light-chain amyloidosis (AL)
WANG He-hua
YAN Mian-sheng
XU Duo-rong
TONG Xiu-zhen
CHEN Wen-fang
LI Juan
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.
Keywords：immunoglobulin light-chain amyloidosissurvival
Publication Date：2009-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：3( 1003-1005 )