伴单克隆免疫球蛋白IgA沉积的膜增生性肾小球肾炎

Recurrent purpura,renal dysfunction with monoclonal IgA-λ deposition

中年男性患者,反复发作双下肢皮肤紫癜16年,肾脏损害表现为肾病综合征,大量镜下血尿,血压及血清肌酐升高,轻度贫血。2009年曾行肾活检诊断为“过敏性紫癜性肾炎”;2016年5月发现血M蛋白阳性(λ-IgA),重复肾活检为膜增生性肾小球肾炎,免疫荧光提示IgA++,轻链染色λ++、κ-,电镜下见晶格状电子致密物沉积;最终诊断为浆细胞病,伴单克隆λ-IgA沉积的膜增性肾小球肾炎,予沙利度胺治疗效果欠佳,硼替佐米治疗后病情好转。

The clinical manifestation of a middle-aged male patient was the paroxysmal rash of the lower limbs,nephrotic syndrome with massive of microscopic hematuria,elevated blood pressure,increased serum creatinine,and mild anemia.First renal biopsy performed in 2009 indicated“Henoch-Schonle in purpura”nephritis.Monoclonal λ-IgA was detected in 2016,and a repeat renal biopsy revealed membranoproliferative glomerulonephritis.Immunofluorescence indicated IgA++,light chain staining λ++,κ-,crystal lattice deposition of electron dense could be seen in the electron microscopy.The final diagnosis was plasma cell disease,proliferative glomerulonephritis with monoclonal IgA deposition.Thalidomide treatment was performed and ineffective,but bortezomib was documented to be medically effective.