An Interesting Case of Proteinuria: AL Amyloidosis

Dipti Chand, R. Gosavi, A. Chakraborty, Tejas Kungar, Yash Paliwal
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Abstract

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 recognise 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. We describe a case of a 54--year-old male patient with monoclonal gammopathy with AL amyloidosis.
一个有趣的蛋白尿病例:AL淀粉样变
轻链(AL)淀粉样变性是最常见的系统性淀粉样变性,与潜在的浆细胞病变有关。这种疾病通常难以识别,因为它的表现范围很广,而症状往往很模糊。临床症状包括肾性蛋白尿伴或不伴肾功能障碍、肝肿大、充血性心力衰竭和自主神经或感觉神经病变。最近的诊断和预后进展包括血清游离轻链测定、心脏磁共振成像和血清学心脏生物标志物。在过去十年中发展的治疗策略是延长这种疾病患者的生存期和保持器官功能。本文综述了AL淀粉样变的诊断、疾病严重程度评估和治疗方法。我们报告一例54岁男性单克隆伽玛病合并AL淀粉样变的病例。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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