肾脏病理在克隆性肾脏病意义上变化很大。在本报告中，我们介绍了一个二十多岁的女性，患有肾病综合症和克隆免疫球蛋白G kappa（血清和尿液）但没有糖尿病。她有肾病综合征、血液学和结缔组织疾病的家族史。肾活检显示结节性肾小球硬化，肾小球毛细血管充满组织细胞，这些组织细胞强烈为kappa阳性，不是lambda。免疫电子显微镜显示组织细胞已浸润到肾小球亚内皮腔，组织细胞的肿大的溶酶体含有kappa轻链，没有明显的结晶形成。骨髓检查未见恶性；因此，我们诊断这个病例为组织细胞性肾小球病，伴随着免疫球蛋白G-kappa浆细胞异常的非晶质内含物。血液学治疗bortezomib和daratumumab降低她的血清kappa链和蛋白尿水平。诊断后两年，她的肾功能保持正常，尿蛋白水平降至1 g/d，自由轻链比率降至3.1。©2023作者。

The kidney pathology of monoclonal gammopathy of renal significance varies greatly. In this report, we present a woman in her 20s with nephrotic syndrome and monoclonal immunoglobulin G kappa (serum and urine) without diabetes. She had a family history of nephrotic syndrome as well as hematologic and connective tissue disorders. A kidney biopsy showed nodular glomerulosclerosis, with the glomerular capillary full of histiocytes, which were strongly positive for kappa, not lambda. Immunoelectron microscopy revealed that histiocytes had infiltrated the glomerular subendothelial space, and enlarged lysosomes of histiocytes contained kappa light chains, without apparent crystalline formation. Bone marrow examination was negative for malignancy; thus, we diagnosed this case as histiocytic glomerulopathy with noncrystalline inclusion associated with immunoglobulin G-kappa plasma cell dyscrasia. Hematologic treatment with bortezomib and daratumumab decreased her level of serum kappa chain and proteinuria. Two years after diagnosis, her kidney function remained normal, urinary protein level decreased to 1 g/d, and free light-chain ratio decreased to 3.1.© 2023 The Authors.