Sanchorawala Vaishali
Department of Medicine, Section of Hematology/Oncology, Boston University Medical Center, Boston, Massachusetts, USA.
Clin J Am Soc Nephrol. 2006 Nov;1(6):1331-41. doi: 10.2215/CJN.02740806. Epub 2006 Oct 11.
Light-chain (AL) amyloidosis is the most common form of systemic amyloidosis and is associated with an underlying plasma cell dyscrasia. The disease often is difficult to recognize because of its broad range of manifestations and what often are vague symptoms. The clinical syndromes at presentation include nephrotic-range proteinuria with or without renal dysfunction, hepatomegaly, congestive heart failure, and autonomic or sensory neuropathy. Recent diagnostic and prognostic advances include the serum free light-chain assay, cardiac magnetic resonance imaging, and serologic cardiac biomarkers. Treatment strategies that have evolved during the past decade are prolonging survival and preserving organ function in patients with this disease. This review outlines approaches to diagnosis, assessment of disease severity, and treatment of AL amyloidosis.
轻链（AL）淀粉样变性是系统性淀粉样变性最常见的形式，与潜在的浆细胞异常增生有关。由于其表现形式广泛且症状往往模糊不清，该病常常难以识别。就诊时的临床综合征包括伴有或不伴有肾功能不全的肾病范围蛋白尿、肝肿大、充血性心力衰竭以及自主神经或感觉神经病变。近期诊断和预后方面的进展包括血清游离轻链检测、心脏磁共振成像以及血清学心脏生物标志物。在过去十年中发展起来的治疗策略正在延长该病患者的生存期并保护其器官功能。本综述概述了AL淀粉样变性的诊断方法、疾病严重程度评估及治疗。
