Thrombotic Microangiopathy, an Unusual Form of Monoclonal Gammopathy of Renal Significance: Report of 3 Cases and Literature Review

Monoclonal gammopathies result from neoplastic clones of the B-cell lineage and may cause kidney disease by various mechanisms. When the underlying clone does not meet criteria for a malignancy requiring treatment, the paraprotein is called a monoclonal gammopathy of renal significance (MGRS). One r...

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Autores principales:	Edward J. Filippone, Eric D. Newman, Li Li, Rakesh Gulati, John L. Farber
Formato:	article
Lenguaje:	EN
Publicado:	Frontiers Media S.A. 2021
Materias:	thrombotic microangiopathy monoclonal gammopathy of renal significance eculizumab plasma cell dyscrasia C3 glomerulopathies alternate pathway of complement Immunologic diseases. Allergy RC581-607
Acceso en línea:	https://doaj.org/article/91d62179090740eab76980a4e0f6a9ab
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spelling	oai:doaj.org-article:91d62179090740eab76980a4e0f6a9ab2021-11-10T07:11:00ZThrombotic Microangiopathy, an Unusual Form of Monoclonal Gammopathy of Renal Significance: Report of 3 Cases and Literature Review1664-322410.3389/fimmu.2021.780107https://doaj.org/article/91d62179090740eab76980a4e0f6a9ab2021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fimmu.2021.780107/fullhttps://doaj.org/toc/1664-3224Monoclonal gammopathies result from neoplastic clones of the B-cell lineage and may cause kidney disease by various mechanisms. When the underlying clone does not meet criteria for a malignancy requiring treatment, the paraprotein is called a monoclonal gammopathy of renal significance (MGRS). One rarely reported kidney lesion associated with benign paraproteins is thrombotic microangiopathy (TMA), provisionally considered as a combination signifying MGRS. Such cases may lack systemic features of TMA, such as a microangiopathic hemolytic anemia, and the disease may be kidney limited. There is no direct deposition of the paraprotein in the kidney, and the presumed mechanism is disordered complement regulation. We report three cases of kidney limited TMA associated with benign paraproteins that had no other detectable cause for the TMA, representing cases of MGRS. Two of the cases are receiving clone directed therapy, and none are receiving eculizumab. We discuss in detail the pathophysiological basis for this possible association. Our approach to therapy involves first ruling out other causes of TMA as well as an underlying B-cell malignancy that would necessitate direct treatment. Otherwise, clone directed therapy should be considered. If refractory to such therapy or the disease is severe and multisystemic, C5 inhibition (eculizumab or ravulizumab) may be indicated as well.Edward J. FilipponeEric D. NewmanLi LiRakesh GulatiJohn L. FarberFrontiers Media S.A.articlethrombotic microangiopathymonoclonal gammopathy of renal significanceeculizumabplasma cell dyscrasiaC3 glomerulopathiesalternate pathway of complementImmunologic diseases. AllergyRC581-607ENFrontiers in Immunology, Vol 12 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	thrombotic microangiopathy monoclonal gammopathy of renal significance eculizumab plasma cell dyscrasia C3 glomerulopathies alternate pathway of complement Immunologic diseases. Allergy RC581-607
spellingShingle	thrombotic microangiopathy monoclonal gammopathy of renal significance eculizumab plasma cell dyscrasia C3 glomerulopathies alternate pathway of complement Immunologic diseases. Allergy RC581-607 Edward J. Filippone Eric D. Newman Li Li Rakesh Gulati John L. Farber Thrombotic Microangiopathy, an Unusual Form of Monoclonal Gammopathy of Renal Significance: Report of 3 Cases and Literature Review
description	Monoclonal gammopathies result from neoplastic clones of the B-cell lineage and may cause kidney disease by various mechanisms. When the underlying clone does not meet criteria for a malignancy requiring treatment, the paraprotein is called a monoclonal gammopathy of renal significance (MGRS). One rarely reported kidney lesion associated with benign paraproteins is thrombotic microangiopathy (TMA), provisionally considered as a combination signifying MGRS. Such cases may lack systemic features of TMA, such as a microangiopathic hemolytic anemia, and the disease may be kidney limited. There is no direct deposition of the paraprotein in the kidney, and the presumed mechanism is disordered complement regulation. We report three cases of kidney limited TMA associated with benign paraproteins that had no other detectable cause for the TMA, representing cases of MGRS. Two of the cases are receiving clone directed therapy, and none are receiving eculizumab. We discuss in detail the pathophysiological basis for this possible association. Our approach to therapy involves first ruling out other causes of TMA as well as an underlying B-cell malignancy that would necessitate direct treatment. Otherwise, clone directed therapy should be considered. If refractory to such therapy or the disease is severe and multisystemic, C5 inhibition (eculizumab or ravulizumab) may be indicated as well.
format	article
author	Edward J. Filippone Eric D. Newman Li Li Rakesh Gulati John L. Farber
author_facet	Edward J. Filippone Eric D. Newman Li Li Rakesh Gulati John L. Farber
author_sort	Edward J. Filippone
title	Thrombotic Microangiopathy, an Unusual Form of Monoclonal Gammopathy of Renal Significance: Report of 3 Cases and Literature Review
title_short	Thrombotic Microangiopathy, an Unusual Form of Monoclonal Gammopathy of Renal Significance: Report of 3 Cases and Literature Review
title_full	Thrombotic Microangiopathy, an Unusual Form of Monoclonal Gammopathy of Renal Significance: Report of 3 Cases and Literature Review
title_fullStr	Thrombotic Microangiopathy, an Unusual Form of Monoclonal Gammopathy of Renal Significance: Report of 3 Cases and Literature Review
title_full_unstemmed	Thrombotic Microangiopathy, an Unusual Form of Monoclonal Gammopathy of Renal Significance: Report of 3 Cases and Literature Review
title_sort	thrombotic microangiopathy, an unusual form of monoclonal gammopathy of renal significance: report of 3 cases and literature review
publisher	Frontiers Media S.A.
publishDate	2021
url	https://doaj.org/article/91d62179090740eab76980a4e0f6a9ab
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Thrombotic Microangiopathy, an Unusual Form of Monoclonal Gammopathy of Renal Significance: Report of 3 Cases and Literature Review

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