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Nephrotic syndrome secondary to proliferative glomerulonephritis with monoclonal immunoglobulin deposits of lambda light chain.

Yun S, Braunhut BL, Walker CN, Bhati W, Sussman AN, Anwer F - Case Rep Nephrol (2014)

Bottom Line: We describe a rare case of a 46-year-old woman with history of refractory nephrotic syndrome and hypertension who presented with worsening proteinuria and kidney function.Kidney biopsy demonstrated glomerular sclerotic change with lambda light chain deposits in the subendothelial space, which is consistent with proliferative glomerulonephritis with monoclonal immunoglobulin deposit (PGNMID).The patient was treated with bortezomib and dexamethasone without clinical improvement and eventually became hemodialysis dependent.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, University of Arizona, Tucson, AZ 85721, USA ; Department of Medicine, Arizona Health Sciences Center, 6th Floor, Room 6336, 1501 N. Campbell Avenue,Tucson, AZ 85719, USA.

ABSTRACT
We describe a rare case of a 46-year-old woman with history of refractory nephrotic syndrome and hypertension who presented with worsening proteinuria and kidney function. Work-up for both autoimmune and infectious diseases and hematologic malignancies including multiple myeloma were negative. Kidney biopsy demonstrated glomerular sclerotic change with lambda light chain deposits in the subendothelial space, which is consistent with proliferative glomerulonephritis with monoclonal immunoglobulin deposit (PGNMID). The patient was treated with bortezomib and dexamethasone without clinical improvement and eventually became hemodialysis dependent.

No MeSH data available.


Related in: MedlinePlus

Laboratory values during the chemotherapy. 16-week treatment with cyclosporine followed by prednisone failed to prevent disease progression. Patient received a total of 4 cycles of bortezomib and dexamethasone, however, with no clinical or laboratory improvement. Disease progressed and patient eventually became hemodialysis dependent.
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fig1: Laboratory values during the chemotherapy. 16-week treatment with cyclosporine followed by prednisone failed to prevent disease progression. Patient received a total of 4 cycles of bortezomib and dexamethasone, however, with no clinical or laboratory improvement. Disease progressed and patient eventually became hemodialysis dependent.

Mentions: A 46-year-old woman with history of nephrotic syndrome was referred from an outside hospital for further evaluation and management of recurrent and progressively worsening nephrotic syndrome with stage III chronic kidney disease (CKD). Kidney biopsy and immunofluorescence (IF) staining one year ago had shown focal segmental proliferative glomerulonephritis and the patient had been treated with 16 weeks of cyclosporine followed by prednisone. Unfortunately, one year later, she presented with recurrent nephrotic symptoms including elevated serum creatinine, hypoalbuminemia, and significant proteinuria (Figure 1).


Nephrotic syndrome secondary to proliferative glomerulonephritis with monoclonal immunoglobulin deposits of lambda light chain.

Yun S, Braunhut BL, Walker CN, Bhati W, Sussman AN, Anwer F - Case Rep Nephrol (2014)

Laboratory values during the chemotherapy. 16-week treatment with cyclosporine followed by prednisone failed to prevent disease progression. Patient received a total of 4 cycles of bortezomib and dexamethasone, however, with no clinical or laboratory improvement. Disease progressed and patient eventually became hemodialysis dependent.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4129672&req=5

fig1: Laboratory values during the chemotherapy. 16-week treatment with cyclosporine followed by prednisone failed to prevent disease progression. Patient received a total of 4 cycles of bortezomib and dexamethasone, however, with no clinical or laboratory improvement. Disease progressed and patient eventually became hemodialysis dependent.
Mentions: A 46-year-old woman with history of nephrotic syndrome was referred from an outside hospital for further evaluation and management of recurrent and progressively worsening nephrotic syndrome with stage III chronic kidney disease (CKD). Kidney biopsy and immunofluorescence (IF) staining one year ago had shown focal segmental proliferative glomerulonephritis and the patient had been treated with 16 weeks of cyclosporine followed by prednisone. Unfortunately, one year later, she presented with recurrent nephrotic symptoms including elevated serum creatinine, hypoalbuminemia, and significant proteinuria (Figure 1).

Bottom Line: We describe a rare case of a 46-year-old woman with history of refractory nephrotic syndrome and hypertension who presented with worsening proteinuria and kidney function.Kidney biopsy demonstrated glomerular sclerotic change with lambda light chain deposits in the subendothelial space, which is consistent with proliferative glomerulonephritis with monoclonal immunoglobulin deposit (PGNMID).The patient was treated with bortezomib and dexamethasone without clinical improvement and eventually became hemodialysis dependent.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, University of Arizona, Tucson, AZ 85721, USA ; Department of Medicine, Arizona Health Sciences Center, 6th Floor, Room 6336, 1501 N. Campbell Avenue,Tucson, AZ 85719, USA.

ABSTRACT
We describe a rare case of a 46-year-old woman with history of refractory nephrotic syndrome and hypertension who presented with worsening proteinuria and kidney function. Work-up for both autoimmune and infectious diseases and hematologic malignancies including multiple myeloma were negative. Kidney biopsy demonstrated glomerular sclerotic change with lambda light chain deposits in the subendothelial space, which is consistent with proliferative glomerulonephritis with monoclonal immunoglobulin deposit (PGNMID). The patient was treated with bortezomib and dexamethasone without clinical improvement and eventually became hemodialysis dependent.

No MeSH data available.


Related in: MedlinePlus