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2018 ; 8
(1
): 70-75
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Proliferative Glomerulonephritis with Monoclonal Immunoglobulin G Lambda
Deposits: Report of the First Pediatric Case
#MMPMID29850460
Torrealba J
; Gattineni J
; Hendricks AR
Case Rep Nephrol Dial
2018[Jan]; 8
(1
): 70-75
PMID29850460
show ga
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 guide accurate disease classification, prognosis, and
treatment.