Abstract:

A 49yearold woman presented with renal insufficiency,proteinuria，hematuria and serum monoclonal immunoglobulin IgGλ.Bone marrow aspirate revealed 8％ plasma cells．Renal biopsy showed mild to moderate mesangial proliferation with positive Congo red staining in the vessels.Immunoflurerscence indicated linear staining along GBMs,TBMs and mesangium for λ light chains and IgG.Ultrastructral examination showed granular electron dense deposits delineating the outer aspect of the TBMs and the endothelial aspect of the GBMs．The final diagnosis was lightheavy chain deposition disease (IgGλ) with light chain amyloidosis,with kidney,skin and heart involved．