关键词: 皮肤紫癜, &lambda, -IgA单克降免疫球蛋白, 膜增性肾小球肾炎

Abstract:

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.

Key words: purpura, monoclonal IgA-λ, membranoproliferative glomerulonephritis