Underlying IgM heavy chain amyloidosis in treatment-refractory IgA nephropathy: A case report  

作　　者：Hai-Ting Wu Yu-Bing Wen Wei Ye Bing-Yan Liu Kai-Ni Shen Rui-Tong Gao Ming-Xi Li 

机构地区：[1]Department of Nephrology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100730,China [2]Department of Hematology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100730,China

出　　处：《World Journal of Clinical Cases》2019年第19期3055-3061,共7页世界临床病例杂志

摘　　要：BACKGROUND Monoclonal immunoglobulin can cause renal damage,with a wide spectrum of pathological changes and clinical manifestations without hematological evidence of malignancy.These disorders can be missed,especially when combined with other kidney diseases.CASE SUMMARY A 61-year-old woman presented with moderate proteinuria with normal renal function.She was diagnosed with IgA nephropathy combined with monoclonal gammopathy of undetermined significance after the first renal biopsy.Although having received immunosuppressive treatment for 3 years,the patient developed nephrotic syndrome.Repeated renal biopsy and laser microdissection/mass spectrometry analysis confirmed heavy chain amyloidosis.After nine cycles of bortezomib,cyclophosphamide and dexamethasone,she achieved very good partial hematological and kidney responses.CONCLUSION Renal injury should be monitored closely in monoclonal gammopathy patients without obvious hematological malignancy,especially in patients with other preexisting renal diseases.BACKGROUND Monoclonal immunoglobulin can cause renal damage, with a wide spectrum of pathological changes and clinical manifestations without hematological evidence of malignancy. These disorders can be missed, especially when combined with other kidney diseases.CASE SUMMARY A 61-year-old woman presented with moderate proteinuria with normal renal function. She was diagnosed with IgA nephropathy combined with monoclonal gammopathy of undetermined significance after the first renal biopsy. Although having received immunosuppressive treatment for 3 years, the patient developed nephrotic syndrome. Repeated renal biopsy and laser microdissection/mass spectrometry analysis confirmed heavy chain amyloidosis. After nine cycles of bortezomib, cyclophosphamide and dexamethasone, she achieved very good partial hematological and kidney responses.CONCLUSION Renal injury should be monitored closely in monoclonal gammopathy patients without obvious hematological malignancy, especially in patients with other preexisting renal diseases.

关 键 词：MONOCLONAL GAMMOPATHY IgA NEPHROPATHY Renal BIOPSY Laser microdissection/mass spectrometry Case report 

分 类 号：R69[医药卫生—泌尿科学]