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A case of podocytic infolding glomerulopathy with multiple myeloma

机译:一例多囊性骨髓瘤的后囊性肾小球病变

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Background Podocytic infolding glomerulopathy (PIG) is a recently described condition causing rare pathological changes to the glomeruli, and has attracted considerable attention. PIG is characterized by specific changes to the thickened glomerular basement membrane (GBM), including microspheres, microtubular structures, and podocytic infolding. Only a small number of cases of PIG have been reported. The clinical features and pathogenesis of this condition are still unclear. To elucidate the characteristics of this glomerulopathy, it is necessary to accumulate information from reported cases. We present here the first reported case of PIG with multiple myeloma. Case presentation A 79-year-old Japanese man was admitted to his local hospital with proteinuria, hypergammaglobulinemia, hypoalbuminemia, and kidney dysfunction. Laboratory tests revealed monoclonal IgG(λ) M proteins in the serum and Bence-Jones proteins in the urine. Bone marrow aspiration showed monoclonal plasma cell proliferation, indicating a diagnosis of multiple myeloma. Renal biopsy was performed to determine the cause of the proteinuria and kidney dysfunction. Histological examination of the biopsy specimen showed glomeruli with an irregularly thickened GBM and bubble-like structures in the capillary walls. Immunofluorescence staining did not show glomerular deposition of immunoglobulins, light chains, or complement components. Congo red staining did not show amyloid deposition. Electron microscopy showed an irregularly thickened GBM with unusual structures in the glomerular capillary walls including podocytic infolding and microspheres, suggesting PIG. There were no electron-dense deposits in the GBM, while various findings indicating podocyte injury were detected. Conclusion We present here the first reported case of PIG in a patient with multiple myeloma. The mechanisms underlying the development of PIG in multiple myeloma are unknown, but may be associated with podocyte injury.
机译:背景技术肾小球内陷性肾小球病(PIG)是一种最近描述的疾病,可引起肾小球罕见的病理变化,并引起了广泛的关注。 PIG的特征是肾小球基底膜增厚(GBM)发生特定变化,包括微球,微管结构和足细胞折叠。仅报告了少数PIG病例。此病的临床特征和发病机制仍不清楚。为了阐明这种肾小球病的特征,有必要积累报告病例的信息。我们在这里介绍第一例多发性骨髓瘤的PIG病例。病例介绍一名79岁的日本男子因蛋白尿,高球蛋白血症,低白蛋白血症和肾功能不全被送往当地医院。实验室测试显示血清中有单克隆IgG(λ)M蛋白,尿中有Bence-Jones蛋白。骨髓穿刺显示单克隆浆细胞增生,表明诊断为多发性骨髓瘤。进行肾脏活检以确定蛋白尿和肾功能不全的原因。活检标本的组织学检查显示,肾小球的GBM呈不规则增厚,毛细血管壁上有气泡状结构。免疫荧光染色未显示免疫球蛋白,轻链或补体成分的肾小球沉积。刚果红染色未显示淀粉样蛋白沉积。电子显微镜显示肾小球毛细血管壁的GBM呈不规则增厚结构,具有异常结构,包括胞囊性折叠和微球体,提示为PIG。 GBM中没有电子致密的沉积物,而各种发现表明足细胞有损伤。结论我们这里是多发性骨髓瘤患者中首例报道的PIG病例。多发性骨髓瘤中PIG发生的潜在机制尚不清楚,但可能与足细胞损伤有关。

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