ANCA-Associated Vasculitides and Hematologic Malignancies: Lessons from the Past and Future Perspectives

The purpose of this paper is to collect and summarize all evidences relating to an association between ANCA-associated vasculitides (AAVs) and hematologic malignancies, in the form of either a paraneoplastic vasculitis or leukemias and lymphomas developing on a preexisting vasculitis. Additionally,...

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Main Authors: Marco Folci, Giacomo Ramponi, Dana Shiffer, Aurora Zumbo, Michele Agosti, Enrico Brunetta
Format: Article
Language:English
Published: Wiley 2019-01-01
Series:Journal of Immunology Research
Online Access:http://dx.doi.org/10.1155/2019/1732175
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author Marco Folci
Giacomo Ramponi
Dana Shiffer
Aurora Zumbo
Michele Agosti
Enrico Brunetta
author_facet Marco Folci
Giacomo Ramponi
Dana Shiffer
Aurora Zumbo
Michele Agosti
Enrico Brunetta
author_sort Marco Folci
collection DOAJ
description The purpose of this paper is to collect and summarize all evidences relating to an association between ANCA-associated vasculitides (AAVs) and hematologic malignancies, in the form of either a paraneoplastic vasculitis or leukemias and lymphomas developing on a preexisting vasculitis. Additionally, the role of cyclophosphamide in vasculitis treatment has been assessed and compared to rituximab. Paraneoplastic AAV seems to be an uncommon presentation of hemopathies. Hematologic malignancy risk in AAV is more likely to be increased by cyclophosphamide, although not yet definitely proven. Furthermore, the pathogenesis of ANCA-associated vasculitis has been reviewed with particular emphasis on the role of proteinase 3 (PR3) in fuelling granulomatosis with polyangiitis (GPA) inflammation. PR3 is a bactericidal protein expressed by neutrophilic granules and on their plasma membrane. Derangements in its expression and function have been linked to leukemias and GPA alike. PR3-derived PR1 peptide is being studied as an immunotherapy target in leukemia and multiple myeloma. This study is aimed at bringing together various evidences from the field of immunological and hematological research, at exposing contradictions, and at revealing novel insights on the association between ANCA-associated vasculitis and hematologic malignancies.
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institution Kabale University
issn 2314-8861
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publishDate 2019-01-01
publisher Wiley
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series Journal of Immunology Research
spelling doaj-art-c97b6a19e5bb48699a16efd3fb72e4862025-02-03T05:50:54ZengWileyJournal of Immunology Research2314-88612314-71562019-01-01201910.1155/2019/17321751732175ANCA-Associated Vasculitides and Hematologic Malignancies: Lessons from the Past and Future PerspectivesMarco Folci0Giacomo Ramponi1Dana Shiffer2Aurora Zumbo3Michele Agosti4Enrico Brunetta5Department of Biomedical Sciences, Humanitas University, Milan, ItalyInternal Medicine, Humanitas Research Hospital, Milan, ItalyDepartment of Biomedical Sciences, Humanitas University, Milan, ItalyDepartment of Biomedical Sciences, Humanitas University, Milan, ItalyUniversity of Milan, Milan, ItalyDepartment of Biomedical Sciences, Humanitas University, Milan, ItalyThe purpose of this paper is to collect and summarize all evidences relating to an association between ANCA-associated vasculitides (AAVs) and hematologic malignancies, in the form of either a paraneoplastic vasculitis or leukemias and lymphomas developing on a preexisting vasculitis. Additionally, the role of cyclophosphamide in vasculitis treatment has been assessed and compared to rituximab. Paraneoplastic AAV seems to be an uncommon presentation of hemopathies. Hematologic malignancy risk in AAV is more likely to be increased by cyclophosphamide, although not yet definitely proven. Furthermore, the pathogenesis of ANCA-associated vasculitis has been reviewed with particular emphasis on the role of proteinase 3 (PR3) in fuelling granulomatosis with polyangiitis (GPA) inflammation. PR3 is a bactericidal protein expressed by neutrophilic granules and on their plasma membrane. Derangements in its expression and function have been linked to leukemias and GPA alike. PR3-derived PR1 peptide is being studied as an immunotherapy target in leukemia and multiple myeloma. This study is aimed at bringing together various evidences from the field of immunological and hematological research, at exposing contradictions, and at revealing novel insights on the association between ANCA-associated vasculitis and hematologic malignancies.http://dx.doi.org/10.1155/2019/1732175
spellingShingle Marco Folci
Giacomo Ramponi
Dana Shiffer
Aurora Zumbo
Michele Agosti
Enrico Brunetta
ANCA-Associated Vasculitides and Hematologic Malignancies: Lessons from the Past and Future Perspectives
Journal of Immunology Research
title ANCA-Associated Vasculitides and Hematologic Malignancies: Lessons from the Past and Future Perspectives
title_full ANCA-Associated Vasculitides and Hematologic Malignancies: Lessons from the Past and Future Perspectives
title_fullStr ANCA-Associated Vasculitides and Hematologic Malignancies: Lessons from the Past and Future Perspectives
title_full_unstemmed ANCA-Associated Vasculitides and Hematologic Malignancies: Lessons from the Past and Future Perspectives
title_short ANCA-Associated Vasculitides and Hematologic Malignancies: Lessons from the Past and Future Perspectives
title_sort anca associated vasculitides and hematologic malignancies lessons from the past and future perspectives
url http://dx.doi.org/10.1155/2019/1732175
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