Proliferative Glomerulonephritis with Monoclonal Immunoglobulin G Lambda Deposits: Report of the First Pediatric Case

Case Reports in Nephrology and Dialysis
Jose TorrealbaAllen R Hendricks

Abstract

Proliferative glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID) is a recently described, uncommon renal disorder which is considered a monoclonal gammopathy of renal significance. Although some patients will have a detectable monoclonal spike, overt hematologic malignancy is found in only a minority. Most patients with PGNMID are over the age of 50 years, and to our knowledge no cases have been reported in children or adolescents. Renal biopsy shows variable histologic patterns by light microscopy, with membranoproliferative and membranous patterns being most common. Immunofluorescence microscopy demonstrates restriction to a single immunoglobulin G heavy chain isotype and a single light chain subtype. Electron microscopy reveals granular, unorganized deposits. We report a rare pediatric case which occurred in a 17-year-old female. The rarity of this entity in the adult population has not permitted a standard treatment regimen to be established. Our adolescent patient was treated with multiple treatment regimens including prednisone, mycophenolate mofetil, rituximab, bortezomib, and daratumumab. Our case demonstrates that awareness of this disorder by pediatric nephrologists and pathologists is vital to guid...Continue Reading

References

May 28, 2009·Journal of the American Society of Nephrology : JASN·Samih H NasrVivette D D'Agati
Sep 30, 2010·Clinical Journal of the American Society of Nephrology : CJASN·Samih H NasrVivette D D'Agati
Jun 3, 2011·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Sean J BarbourAlex B Magil
Jun 28, 2011·Clinical Journal of the American Society of Nephrology : CJASN·Elsa GuiardPierre Ronco
Jun 28, 2011·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Alia AlbawardiTibor Nadasdy
Jan 22, 2015·Kidney International·Frank BridouxUNKNOWN International Kidney and Monoclonal Gammopathy Research Group

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Citations

Oct 13, 2020·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Paul MillerMegan L Troxell
May 1, 2021·Journal of Clinical Medicine·Rupali S AvasareJonathan J Hogan

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Methods Mentioned

BETA
biopsy
electrophoresis
urine collection
light microscopy
biopsies

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