Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
Blood Rev. 2012 Jul;26(4):137-54. doi: 10.1016/j.blre.2012.03.001. Epub 2012 Apr 25.
Immunoglobulin light chain (AL) amyloidosis is the most common acquired systemic amyloidoses. Its presentation is often insidious and progressive, which may delay diagnosis. The interval between first symptoms and actual diagnosis along the intrinsic heterogeneity of tissue tropism create a wide spectrum of presentations, both in terms of scope and depth of symptoms and signs and functional status of patients. In this review, the authors review the pathogenesis, diagnosis and differential diagnosis of AL amyloidosis along with the prognosis and state-of-the-art management for patients with this affliction.
免疫球蛋白轻链（AL）淀粉样变性是最常见的获得性系统性淀粉样变性。其表现通常是隐匿和进行性的，这可能会延迟诊断。由于组织趋向性的固有异质性，从首发症状到实际诊断的间隔时间较长，导致临床表现范围广泛，从症状和体征的深度和广度以及患者的功能状态来看都是如此。在这篇综述中，作者回顾了 AL 淀粉样变性的发病机制、诊断和鉴别诊断，以及对这种疾病患者的预后和最新治疗方法。
