Duxbury Hayley J, Lee Vincent W, Kwok Fiona, Viswanathan Seethalakshmi
Tissue Pathology and Diagnostic Oncology, Institute of Clinical Pathology and Medical Research, Westmead Hospital, Westmead, New South Wales, Australia.
Westmead Applied Research Centre, The University of Sydney, Sydney, New South Wales, Australia.
BMJ Case Rep. 2025 Apr 2;18(4):e264201. doi: 10.1136/bcr-2024-264201.
Proliferative glomerulonephritis with monoclonal immunoglobulin deposits is a form of monoclonal gammopathy of renal significance that can rarely co-occur with thrombotic microangiopathy (TMA). We report a rare case in a patient who presented with nephrotic syndrome, in whom the renal biopsy showed TMA and underlying granular electron-dense deposits predominantly along the subendothelial aspect of the glomerular basement membrane and mesangium. Immunofluorescence performed on proteinase-digested formalin-fixed, paraffin-embedded sections showed IgG3 kappa light chain restriction. Further clinical, radiologic and haematologic investigations showed no evidence of any underlying neoplastic B-cell or plasma cell clone. Following multidisciplinary team input, the patient was treated with bortezomib and plasma exchange, allowing long-term clinical stabilisation.
伴有单克隆免疫球蛋白沉积的增殖性肾小球肾炎是一种具有肾脏意义的单克隆丙种球蛋白病，很少与血栓性微血管病（TMA）同时发生。我们报告了一例罕见病例，该患者表现为肾病综合征，肾活检显示TMA以及主要沿肾小球基底膜和系膜内皮下的颗粒状电子致密沉积物。对蛋白酶消化的福尔马林固定、石蜡包埋切片进行免疫荧光检查显示IgG3 κ轻链限制。进一步的临床、放射学和血液学检查未发现任何潜在的肿瘤性B细胞或浆细胞克隆的证据。在多学科团队的参与下，该患者接受了硼替佐米和血浆置换治疗，实现了长期临床稳定。
