Bataille Y, Bovy C, Lancellotti P, Melchior V, Delbecque K, Beguin Y, Krzesinski J M
Department of nephrology and hypertension, University Hospital Liège, Domaine universitaire du Sart Tilman- B.35, B 4000 Liege 1, Belgique.
Acta Clin Belg. 2005 Mar-Apr;60(2):94-7. doi: 10.1179/acb.2005.017.
AL amyloidosis is a rare systemic disease resulting from tissue accumulation of amyloid fibrils derived from monoclonal immunoglobulin light chains. It can disrupt the tissue architecture and consequently cause organ dysfunction. The prognosis is poor with a median survival of 13 months in untreated patients. By illustrating the case of a patient whose AL amyloidosis was detected after presenting a nephrotic syndrome, the characteristics of the disease are reviewed as well as diagnostic criteria and current available therapeutics.
轻链型淀粉样变性是一种罕见的全身性疾病，由单克隆免疫球蛋白轻链衍生的淀粉样纤维在组织中蓄积所致。它可破坏组织结构，进而导致器官功能障碍。未经治疗的患者预后较差，中位生存期为13个月。通过阐述一名在出现肾病综合征后被诊断为轻链型淀粉样变性患者的病例，对该疾病的特点、诊断标准及当前可用的治疗方法进行了综述。
