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Monoclonal Gammopathies of Renal Significance: Renal Biopsy and Beyond

Monoclonal Gammopathies of Renal Significance (MGRS) are a rather heterogeneous group of renal disorders caused by a circulating monoclonal (MC) immunoglobulin (Ig) component, often in the absence of multiple myeloma (MM) or another clinically relevant lymphoproliferative disorder. Nevertheless, sub...

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Autores principales: Menè, Paolo, De Alexandris, Lorenzo, Moioli, Alessandra, Raffa, Salvatore, Stoppacciaro, Antonella
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7407125/
https://www.ncbi.nlm.nih.gov/pubmed/32629844
http://dx.doi.org/10.3390/cancers12071741
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author Menè, Paolo
De Alexandris, Lorenzo
Moioli, Alessandra
Raffa, Salvatore
Stoppacciaro, Antonella
author_facet Menè, Paolo
De Alexandris, Lorenzo
Moioli, Alessandra
Raffa, Salvatore
Stoppacciaro, Antonella
author_sort Menè, Paolo
collection PubMed
description Monoclonal Gammopathies of Renal Significance (MGRS) are a rather heterogeneous group of renal disorders caused by a circulating monoclonal (MC) immunoglobulin (Ig) component, often in the absence of multiple myeloma (MM) or another clinically relevant lymphoproliferative disorder. Nevertheless, substantial kidney damage could occur, despite the “benign” features of the bone-marrow biopsy. One example is renal amyloidosis, often linked to a small clone of plasma cells, without the invasive features of MM. However, patients with amyloidosis may present with a nephrotic syndrome and renal failure, eventually leading to end-stage kidney disease. At the same time, other organs, such as the heart and the liver, may be severely damaged by Ig light chains or amyloid deposits, occasionally resulting in fatal arrhythmias and/or organ failure. Acute kidney injury (AKI) may as well result from massive excretion of MC proteins, with deposition disease in glomeruli or renal tubules, not rarely obstructed by luminal aggregates, or “casts”. Proliferative glomerulonephritis with monoclonal Ig deposits is another, less frequent clinical presentation of an MGRS. The present review deals with the implications of MGRS for renal function and prognosis, and the potential of tools, such as the renal biopsy, for assessing clinical risk and guiding therapy of the underlying condition.
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spelling pubmed-74071252020-08-11 Monoclonal Gammopathies of Renal Significance: Renal Biopsy and Beyond Menè, Paolo De Alexandris, Lorenzo Moioli, Alessandra Raffa, Salvatore Stoppacciaro, Antonella Cancers (Basel) Review Monoclonal Gammopathies of Renal Significance (MGRS) are a rather heterogeneous group of renal disorders caused by a circulating monoclonal (MC) immunoglobulin (Ig) component, often in the absence of multiple myeloma (MM) or another clinically relevant lymphoproliferative disorder. Nevertheless, substantial kidney damage could occur, despite the “benign” features of the bone-marrow biopsy. One example is renal amyloidosis, often linked to a small clone of plasma cells, without the invasive features of MM. However, patients with amyloidosis may present with a nephrotic syndrome and renal failure, eventually leading to end-stage kidney disease. At the same time, other organs, such as the heart and the liver, may be severely damaged by Ig light chains or amyloid deposits, occasionally resulting in fatal arrhythmias and/or organ failure. Acute kidney injury (AKI) may as well result from massive excretion of MC proteins, with deposition disease in glomeruli or renal tubules, not rarely obstructed by luminal aggregates, or “casts”. Proliferative glomerulonephritis with monoclonal Ig deposits is another, less frequent clinical presentation of an MGRS. The present review deals with the implications of MGRS for renal function and prognosis, and the potential of tools, such as the renal biopsy, for assessing clinical risk and guiding therapy of the underlying condition. MDPI 2020-06-30 /pmc/articles/PMC7407125/ /pubmed/32629844 http://dx.doi.org/10.3390/cancers12071741 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Menè, Paolo
De Alexandris, Lorenzo
Moioli, Alessandra
Raffa, Salvatore
Stoppacciaro, Antonella
Monoclonal Gammopathies of Renal Significance: Renal Biopsy and Beyond
title Monoclonal Gammopathies of Renal Significance: Renal Biopsy and Beyond
title_full Monoclonal Gammopathies of Renal Significance: Renal Biopsy and Beyond
title_fullStr Monoclonal Gammopathies of Renal Significance: Renal Biopsy and Beyond
title_full_unstemmed Monoclonal Gammopathies of Renal Significance: Renal Biopsy and Beyond
title_short Monoclonal Gammopathies of Renal Significance: Renal Biopsy and Beyond
title_sort monoclonal gammopathies of renal significance: renal biopsy and beyond
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7407125/
https://www.ncbi.nlm.nih.gov/pubmed/32629844
http://dx.doi.org/10.3390/cancers12071741
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