The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement
The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement
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DOI：
10.1093/qjmed/91.2.141
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发表时间：
1998-02-01
期刊：
影响因子：
--
通讯作者：
Falk, RH
中科院分区：
文献类型：
--
作者：
Dubrey, SW;Cha, K;Falk, RH
我们回顾了232例原发性心脏淀粉样变性患者的临床表现、检查、治疗、预后和转归。其中男性142人，女性90人。就诊时的中位年龄为59岁（范围29 - 85岁）。AL心脏病在40岁以下患者（3.0%）和非高加索人（6.5%）中均不常见。疲劳和虚弱是最常见的症状。眶周瘀斑和巨舌症的特征分别为12.5%和27.2%。AL心脏淀粉样变性是不常见的孤立（3.9%），最常见的患者有多器官功能障碍的特点，大量蛋白尿和吸收不良的特点在这方面占主导地位。心脏受累是最差的预后指标，从诊断开始的中位生存期为1.08年，随着心力衰竭的发生而下降到0.75年。目前的治疗方法似乎可以延长生存期，超声心动图显示的左室壁厚度、质量和射血分数以及信号平均心电图显示的晚电位可用于预后分层。AL淀粉样变性的心脏受累是迅速致命的。在超声心动图显示室壁增厚、心腔大小正常、EKG电压低和有证据提示多系统疾病的心力衰竭患者中，应怀疑该疾病。
We reviewed clinical presentation, investigations, therapy, prognosis and outcome of 232 patients with primary (AL) cardiac amyloidosis. There were 142 men and 90 women. Median age at presentation was 59 years (range 29-85). AL heart disease was unusual both in patients under the age of 40 (3.0%) and in non-Caucasians (6.5%). Fatigue and weakness were the commonest presenting symptoms. Hallmark features of periorbital ecchymoses and macroglossia were present in 12.5% and 27.2%, respectively. AL cardiac amyloidosis was unusual in isolation (3.9%), and most frequently patients had features of multiorgan dysfunction; heavy proteinuria and features of malabsorption predominating in this respect. Heart involvement represents the worst prognostic indicator, with a median survival from diagnosis of 1.08 years, falling to 0.75 years with the onset of heart failure. Current therapeutic procedures appear to prolong survival, with left ventricular wall thickness, mass and ejection fraction on echocardiography and late potentials on signal averaged electrocardiography of use in prognostic stratification. Cardiac involvement from AL amyloidosis is rapidly fatal. it should be suspected in all patients with heart failure who have wall thickening on echo, normal chamber sizes, low EKG voltages and evidence suggesting a multisystem disease.