Heavy chain deposition disease presenting with nephrotic syndrome as the initial manifestation in an elderly patient:a case report
MEI Jing
LIAO Yuanjiang
SHI Yan
Abstract：Objective To summarize the clinical characteristics of heavy chain deposition disease(HCDD)in an el-derly patient presenting with nephrotic syndrome as the initial manifestation so as to provide a reference for the early clini-cal identification and timely symptomatic treatment of HCDD.Methods A retrospective analysis was conducted on the clinical manifestations,physical examination,laboratory examination,imaging examination and treatment process of an el-derly patient with HCDD who presented with nephrotic syndrome as the initial manifestation.Results A 76-year-old fe-male was admitted to the hospital with cough,shortness of breath,and edema.Laboratory tests revealed anemia,massive proteinuria(24-hour urine protein 4,142 mg),elevated serum creatinine(159 μmol/L),and hypocomplementemia(C3 0.46 g/L,C4 0.06 g/L).Serum immunofixation electrophoresis detected IgGλ-type M protein,and flow cytometry of bone marrow showed 1.48%monoclonal plasma cells.Light microscopy of renal biopsy revealed mesangial nodular sclerosis,and immunofluorescence showed IgG3 deposition along the capillary loops and mesangial areas,while κ and λ light chains were negative.Electron microscopy confirmed granular electron-dense deposits in the mesangial area and the inner side of the glomerular basement membrane,which was consistent with the pathological diagnosis of HCDD.After two courses of bortezomib combined with dexamethasone(BD regimen)treatment,the M protein turned negative,24-hour urine protein decreased to 395.9 mg,serum creatinine returned to 86 μmol/L,and complement C3 and C4 levels increased to 0.57 g/L and 0.17 g/L,respectively.During a 3-year follow-up,the patient's condition remained stable without edema or shortness of breath,and she was able to perform daily activities normally.Conclusion For elderly patients with nephrotic syn-drome,if they have hypocomplementemia and positive M protein,renal biopsy should be performed as soon as possible to confirm HCDD,and a regimen containing proteasome inhibitors should be initiated promptly,which can significantly im-prove long-term prognosis.
Keywords：heavy chain deposition diseasenephrotic syndromeadvanced agebortezomib
Publication Date：2025-12-25
Online Publishing Date：2026-01-12(First online date of this platform, not the publication date of the document)
Pages：4( 91-94 )