Multiple Myeloma Presenting as Acute Kidney Failure Secondary to Lambda Light Chain Deposition

Marie-Eve Emond-Boisjoly, É. Lemieux-Blanchard, Antonia Maietta, Stéphanie Forté
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Abstract

Renal monoclonal immunoglobulin deposition disease (MIDD) is a rare disease defined by deposition of monoclonal light chains and/or heavy chains on basement membranes and vascular walls of the kidney. We describe a case of a 71-year-old woman with kidney failure secondary to monoclonal immunoglobulin deposition disease lambda in association with plasma cell dyscrasia. Her initial serum protein electrophoresis did not demonstrate a monoclonal protein, and classic cast nephropathy was absent on renal biopsy. However, lambda light chain deposits and associated changes confirmed MIDD. She achieved a very good partial response (VGRP) after 8 cycles of CyBorD (cyclophosphamide, bortezomib, dexamethasone) and her kidney function improved. This case highlights the importance of an early diagnostic with renal biopsy to prevent end-stage renal disease. A review of the existing literature and a discussion on the management of the disease is presented.
Lambda轻链沉积继发急性肾功能衰竭的多发性骨髓瘤
肾脏单克隆免疫球蛋白沉积病(MIDD)是一种罕见的疾病,其特征是单克隆轻链和/或重链沉积在肾脏的基底膜和血管壁上。我们描述了一例71岁女性肾衰竭,继发于单克隆免疫球蛋白沉积病lambda并伴有浆细胞功能障碍。她最初的血清蛋白电泳没有显示单克隆蛋白,肾活检中也没有典型的铸造肾病。然而,lambda轻链沉积物和相关变化证实了MIDD。在8个周期的CyBorD(环磷酰胺、硼替佐米、地塞米松)治疗后,她获得了非常好的部分反应(VGRP),肾功能得到改善。该病例强调了肾活检早期诊断对预防终末期肾病的重要性。对现有文献进行了综述,并对该疾病的管理进行了讨论。
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