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肾脏病与透析肾移植杂志

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伴结晶形成的轻链管型肾病

  

  • 出版日期:2020-08-28 发布日期:2020-08-22

Crystalline light chain cast nephropathy

  • Online:2020-08-28 Published:2020-08-22

摘要: 56岁女性患者,临床表现为少尿型急性肾损伤,伴肋骨骨折、贫血,血免疫固定电泳示λ型IgG单克隆免疫球蛋白条带,血尿游离轻链检查示κ/λ比值明显下降,尿本周蛋白阳性,骨髓细胞学示浆细胞比例17%。肾脏病理示伴结晶形成的轻链管型肾病。最终确诊为多发性骨髓瘤DurieSalmon分期Ⅲ期B组,伴结晶形成的轻链管型肾病,急性肾损伤3期。

关键词: 多发性骨髓瘤, 伴结晶形成的轻链管型肾病, 肾活检

Abstract: A 56yearold woman presented with oliguric acute renal failure,rib fracture and anemia.Serum immunofixation electrophoresis identified a monoclonal immunoglobulin (Ig) G and λ protein.The free κ/λ ratio was abnormal and urine BenceJones protein was positive.Bone marrow biopsy revealed plasmocytosis (17%).A kidney biopsy was performed and showed light chain cast nephropathy with crystal formation.Finally,the patient was diagnosed with multiple myeloma (DSS ⅢB),crystalline light chain cast nephropathy and acute kidney injury stage 3.