伴结晶形成的轻链管型肾病

Crystalline light chain cast nephropathy

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。

A 56⁃year⁃old woman presented with oliguric acute renal failure,rib fracture and anemia.Serum immunofixation electrophoresis identified a monoclonal immunoglobulin(Ig)G andλprotein.The freeκ/λratio 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(DSSⅢB),crystalline light chain cast nephropathy and acute kidney injury stage 3.