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Membranoproliferative glomerulonephritis with masked monotypic immunoglobulin deposits

The diagnosis of membranoproliferative glomerulonephritis (MPGN) has recently undergone change from an electron microscopy-based classification scheme to one based largely on immunofluorescence findings. This change is due to the recognition that many of these cases are driven by abnormalities of th...

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Autores principales: Larsen, Christopher P, Messias, Nidia C, Walker, Patrick D, Fidler, Mary E, Cornell, Lynn D, Hernandez, Loren H, Alexander, Mariam P, Sethi, Sanjeev, Nasr, Samih H
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4687465/
https://www.ncbi.nlm.nih.gov/pubmed/26154922
http://dx.doi.org/10.1038/ki.2015.195
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author Larsen, Christopher P
Messias, Nidia C
Walker, Patrick D
Fidler, Mary E
Cornell, Lynn D
Hernandez, Loren H
Alexander, Mariam P
Sethi, Sanjeev
Nasr, Samih H
author_facet Larsen, Christopher P
Messias, Nidia C
Walker, Patrick D
Fidler, Mary E
Cornell, Lynn D
Hernandez, Loren H
Alexander, Mariam P
Sethi, Sanjeev
Nasr, Samih H
author_sort Larsen, Christopher P
collection PubMed
description The diagnosis of membranoproliferative glomerulonephritis (MPGN) has recently undergone change from an electron microscopy-based classification scheme to one based largely on immunofluorescence findings. This change is due to the recognition that many of these cases are driven by abnormalities of the alternative complement cascade, resulting in the concept of C3 glomerulopathy. Here we reviewed our case files to identify those with an MPGN pattern that show false negative staining for monoclonal immunoglobulins by routine immunofluorescence. Monoclonal immunoglobulin deposits were unmasked by performing immunofluorescence on formalin-fixed paraffin embedded tissue after protease digestion. Clinico-pathological details of 16 such cases with a mean serum creatinine of 2.7 mg/dl and mean 24 h proteinuria of 7.1 g were then determined. Hypocomplementemia was present in two-thirds of patients. Fourteen patients had a paraprotein on serum immunofixation, all of which matched the biopsy immunofluorescence staining pattern. Bone marrow biopsy showed plasma cell dyscrasia or B-cell lymphoproliferative disorder in 13 patients. Ten of these patients had findings on biopsy most consistent with C3 glomerulonephritis prior to performing paraffin immunofluorescence. Thus a high index of suspicion is necessary to avoid misdiagnosis in these cases, as many would have been mistakenly diagnosed as C3 glomerulopathy or unclassified MPGN if paraffin immunofluorescence was not performed.
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spelling pubmed-46874652016-01-07 Membranoproliferative glomerulonephritis with masked monotypic immunoglobulin deposits Larsen, Christopher P Messias, Nidia C Walker, Patrick D Fidler, Mary E Cornell, Lynn D Hernandez, Loren H Alexander, Mariam P Sethi, Sanjeev Nasr, Samih H Kidney Int Clinical Investigation The diagnosis of membranoproliferative glomerulonephritis (MPGN) has recently undergone change from an electron microscopy-based classification scheme to one based largely on immunofluorescence findings. This change is due to the recognition that many of these cases are driven by abnormalities of the alternative complement cascade, resulting in the concept of C3 glomerulopathy. Here we reviewed our case files to identify those with an MPGN pattern that show false negative staining for monoclonal immunoglobulins by routine immunofluorescence. Monoclonal immunoglobulin deposits were unmasked by performing immunofluorescence on formalin-fixed paraffin embedded tissue after protease digestion. Clinico-pathological details of 16 such cases with a mean serum creatinine of 2.7 mg/dl and mean 24 h proteinuria of 7.1 g were then determined. Hypocomplementemia was present in two-thirds of patients. Fourteen patients had a paraprotein on serum immunofixation, all of which matched the biopsy immunofluorescence staining pattern. Bone marrow biopsy showed plasma cell dyscrasia or B-cell lymphoproliferative disorder in 13 patients. Ten of these patients had findings on biopsy most consistent with C3 glomerulonephritis prior to performing paraffin immunofluorescence. Thus a high index of suspicion is necessary to avoid misdiagnosis in these cases, as many would have been mistakenly diagnosed as C3 glomerulopathy or unclassified MPGN if paraffin immunofluorescence was not performed. Nature Publishing Group 2015-10 2015-07-08 /pmc/articles/PMC4687465/ /pubmed/26154922 http://dx.doi.org/10.1038/ki.2015.195 Text en Copyright © 2015 International Society of Nephrology http://creativecommons.org/licenses/by-nc-nd/4.0/ This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License. The images or other third party material in this article are included in the article's Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by-nc-nd/4.0/
spellingShingle Clinical Investigation
Larsen, Christopher P
Messias, Nidia C
Walker, Patrick D
Fidler, Mary E
Cornell, Lynn D
Hernandez, Loren H
Alexander, Mariam P
Sethi, Sanjeev
Nasr, Samih H
Membranoproliferative glomerulonephritis with masked monotypic immunoglobulin deposits
title Membranoproliferative glomerulonephritis with masked monotypic immunoglobulin deposits
title_full Membranoproliferative glomerulonephritis with masked monotypic immunoglobulin deposits
title_fullStr Membranoproliferative glomerulonephritis with masked monotypic immunoglobulin deposits
title_full_unstemmed Membranoproliferative glomerulonephritis with masked monotypic immunoglobulin deposits
title_short Membranoproliferative glomerulonephritis with masked monotypic immunoglobulin deposits
title_sort membranoproliferative glomerulonephritis with masked monotypic immunoglobulin deposits
topic Clinical Investigation
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4687465/
https://www.ncbi.nlm.nih.gov/pubmed/26154922
http://dx.doi.org/10.1038/ki.2015.195
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