多发性骨髓瘤合并肾单克隆免疫球蛋白沉积病1例报告

Sham Sunder , Himanshu Verma , Minakshi Bhardwaj , K. Venkataramanan
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摘要

b细胞增殖性疾病可能通过细胞数量增加、这些细胞产生的分子浓度增加或两者兼而有之而表现出致病作用。大多数与单克隆γ球蛋白病相关的肾脏疾病是由单克隆免疫球蛋白或其亚基在肾脏不同腔室的沉积引起的。肾单克隆免疫球蛋白沉积病(MIDD)是指单克隆轻链成分沉积于肾基底膜、系膜,常导致结节性硬化性肾小球病变。肾脏MIDD的临床特征包括蛋白尿伴或不伴肾功能衰竭伴蛋白异常血症。我们描述了一个68岁的男性表现为肿胀,大量蛋白尿和晚期氮血症,其肾活检显示结节性肾小球硬化。患者有骨溶解性病变,尿电泳和骨髓活检浆细胞呈m峰,β2微球蛋白水平升高,具有多发性骨髓瘤的特征。
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An interesting case of multiple myeloma with renal monoclonal immunoglobulin deposition disease – A case report

B-cell proliferative disorders may display the pathogenic effects by increased cell numbers, increased concentration of the molecules produced by these cells or both. Most forms of renal disease associated with monoclonal gammaglobulinopathy results from deposition of monoclonal immunoglobulins or their subunits in different compartment of kidney. Renal monoclonal immunoglobulin deposition disease (MIDD) is defined by deposits of monoclonal light chain components in renal basement membrane, mesangium which often produces a nodular sclerosing glomerulopathy. Clinical features of renal MIDD include proteinuria with or without renal failure with dysproteinemias.

We describe one case of a 68 years old male presenting with swelling with heavy proteinuria and advanced azotaemia whose renal biopsy showed nodular glomerulosclerosis. The patient had features of multiple myeloma as he had bony lytic lesions, M-spike in urine electrophoresis and plasma cells in bone marrow biopsy and elevated β2 microglobulin level.

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