关键词: IgA, 具有肾脏意义的单克隆免疫球蛋白病, 增生性肾小球肾炎, 微管状结构

Abstract:

A 51yearold man presented as recurrent skin purpura rash,proteinuria and hematuria.The first renal biopsy showed mesangioproliferative glomerulonephritis with IgA deposits and a diagnosis of Henoch Schonlein purpura nephritis was made.He developed renal dysfunction and hypertension,as well as extrarenal manifestations of anemia,and monoclonal IgAλ in serum immunofixation electrophoresis,decreased κ∶λ ratio,without cryoglobulinemia.Repeat biopsies revealed membranoproliferative glomerulonephritis (MPGN) with thrombi．Immunoflurerscence indicated IgA and lamda light chain staining in glomeruli．Ultrastructral examination showed presence of electrondense deposition of orgnized microtubules,in  mesangium,subendothelial,intraluminal, intracytoplasmic,and subepithelial area．The final diagnosis was proliferative glomerulonephritis with microtubular monoclonal IgA lamda deposits．

Key words: IgA, monoclonal gammopathy of renal significance, proliferative glomerulonephritis, microtubular structures