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首页> 外文期刊>Kidney Research and Clinical Practice >A case of Fanconi syndrome accompanied by crystal depositions in tubular cells in a patient with multiple myeloma
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A case of Fanconi syndrome accompanied by crystal depositions in tubular cells in a patient with multiple myeloma

机译:多发性骨髓瘤患者的范科尼综合征一例伴有肾小管细胞晶体沉积

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Fanconi syndrome (FS) is a rare condition that is characterized by defects in the proximal tubular function. A 48-year-old woman was admitted for evaluation of proteinuria. The patient showed normal anion gap acidosis, normoglycemic glycosuria, hypophosphatemia, and hypouricemia. Thus, her condition was compatible with FS. The M peak was found behind the beta globulin region in urine protein electrophoresis. Upon bone marrow examination, we found that 24% of cells were CD138+ plasma cells with kappa restriction. From a kidney biopsy, we found crystalline inclusions within proximal tubular epithelial cells. Thereafter, she was diagnosed with FS accompanied by multiple myeloma. The patient received chemotherapy and autologous stem cell transplantation, and obtained very good partial hematologic response. However, proximal tubular dysfunction was persistent until 1 year after autologous stem cell transplantation. In short, we report a case of FS accompanied by multiple myeloma, demonstrating crystalline inclusion in proximal tubular cells on kidney biopsy.
机译:范可尼综合征(FS)是一种罕见病,其特征是近端肾小管功能受损。一名48岁的女性被接纳进行蛋白尿评估。该患者表现出正常的阴离子间隙酸中毒,血糖正常的糖尿,低磷血症和低尿酸血症。因此,她的病情与FS相符。在尿蛋白电泳中,在β球蛋白区域的后面发现了M峰。通过骨髓检查,我们发现24%的细胞是受kappa限制的CD138 +浆细胞。从肾脏活检中,我们发现近端肾小管上皮细胞内有晶体包裹体。此后,她被诊断患有FS并发多发性骨髓瘤。患者接受了化学疗法和自体干细胞移植,并获得了很好的局部血液学应答。然而,近端肾小管功能障碍持续至自体干细胞移植后1年。简而言之,我们报告了一例伴有多发性骨髓瘤的FS病例,表明肾脏活检中近端肾小管细胞中有晶体包涵体。

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