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Unmet Clinical Needs in the Management of Idiopathic Multicentric Castleman Disease: A Consensus-based Position Paper From an ad hoc Expert Panel

Castleman disease describes a group of heterogeneous clinicopathological disorders now included in the tumor-like lesions with B-cell predominance of the World Health Organization classification. Managing idiopathic multicentric Castleman disease (iMCD) is challenging, because few systematic studies...

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Autores principales: Zinzani, Pier Luigi, Paulli, Marco, Arcaini, Luca, Della Torre, Emanuel, Ferrero, Simone, Figuera, Amalia, Frigeri, Ferdinando, Martelli, Maurizio, Sabattini, Elena, Scarpa, Riccardo, Barosi, Giovanni
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10208718/
https://www.ncbi.nlm.nih.gov/pubmed/37234822
http://dx.doi.org/10.1097/HS9.0000000000000891
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author Zinzani, Pier Luigi
Paulli, Marco
Arcaini, Luca
Della Torre, Emanuel
Ferrero, Simone
Figuera, Amalia
Frigeri, Ferdinando
Martelli, Maurizio
Sabattini, Elena
Scarpa, Riccardo
Barosi, Giovanni
author_facet Zinzani, Pier Luigi
Paulli, Marco
Arcaini, Luca
Della Torre, Emanuel
Ferrero, Simone
Figuera, Amalia
Frigeri, Ferdinando
Martelli, Maurizio
Sabattini, Elena
Scarpa, Riccardo
Barosi, Giovanni
author_sort Zinzani, Pier Luigi
collection PubMed
description Castleman disease describes a group of heterogeneous clinicopathological disorders now included in the tumor-like lesions with B-cell predominance of the World Health Organization classification. Managing idiopathic multicentric Castleman disease (iMCD) is challenging, because few systematic studies or comparative randomized clinical trials have been conducted. International, consensus evidence-based guidelines for iMCD were published in 2018, but gaps in the therapeutic options for difficult-to-treat patients, who do not respond to siltuximab and other conventional therapies, still exist. This article presents the results of group discussion among an ad hoc constituted Panel of Italian experts to identify and address unmet clinical needs (UCNs) in managing iMCD. Recommendations on the appropriateness of clinical decisions and proposals for new research concerning the identified UCNs were issued through formalized multiple-step procedures after a comprehensive analysis of the scientific literature. The following key UCNs were addressed: strengthening the diagnostic certainty in iMCD patients before planning first-line therapy; management of siltuximab therapy; choice and management of immune-modulating, or chemotherapy agents in patients resistant/intolerant to siltuximab therapy. While most of the conclusions reached by the Panel are consistent with the existing guidelines, some alternative therapeutic options were stressed, and the discussion contributed to bringing forth the issues that need further investigation. Hopefully, this comprehensive overview will improve the practice of iMCD and inform the design and implementation of new studies in the field.
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spelling pubmed-102087182023-05-25 Unmet Clinical Needs in the Management of Idiopathic Multicentric Castleman Disease: A Consensus-based Position Paper From an ad hoc Expert Panel Zinzani, Pier Luigi Paulli, Marco Arcaini, Luca Della Torre, Emanuel Ferrero, Simone Figuera, Amalia Frigeri, Ferdinando Martelli, Maurizio Sabattini, Elena Scarpa, Riccardo Barosi, Giovanni Hemasphere Guideline Article – Consensus based Castleman disease describes a group of heterogeneous clinicopathological disorders now included in the tumor-like lesions with B-cell predominance of the World Health Organization classification. Managing idiopathic multicentric Castleman disease (iMCD) is challenging, because few systematic studies or comparative randomized clinical trials have been conducted. International, consensus evidence-based guidelines for iMCD were published in 2018, but gaps in the therapeutic options for difficult-to-treat patients, who do not respond to siltuximab and other conventional therapies, still exist. This article presents the results of group discussion among an ad hoc constituted Panel of Italian experts to identify and address unmet clinical needs (UCNs) in managing iMCD. Recommendations on the appropriateness of clinical decisions and proposals for new research concerning the identified UCNs were issued through formalized multiple-step procedures after a comprehensive analysis of the scientific literature. The following key UCNs were addressed: strengthening the diagnostic certainty in iMCD patients before planning first-line therapy; management of siltuximab therapy; choice and management of immune-modulating, or chemotherapy agents in patients resistant/intolerant to siltuximab therapy. While most of the conclusions reached by the Panel are consistent with the existing guidelines, some alternative therapeutic options were stressed, and the discussion contributed to bringing forth the issues that need further investigation. Hopefully, this comprehensive overview will improve the practice of iMCD and inform the design and implementation of new studies in the field. Lippincott Williams & Wilkins 2023-05-23 /pmc/articles/PMC10208718/ /pubmed/37234822 http://dx.doi.org/10.1097/HS9.0000000000000891 Text en Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Hematology Association. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND) (https://creativecommons.org/licenses/by-nc-nd/4.0/) , where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.
spellingShingle Guideline Article – Consensus based
Zinzani, Pier Luigi
Paulli, Marco
Arcaini, Luca
Della Torre, Emanuel
Ferrero, Simone
Figuera, Amalia
Frigeri, Ferdinando
Martelli, Maurizio
Sabattini, Elena
Scarpa, Riccardo
Barosi, Giovanni
Unmet Clinical Needs in the Management of Idiopathic Multicentric Castleman Disease: A Consensus-based Position Paper From an ad hoc Expert Panel
title Unmet Clinical Needs in the Management of Idiopathic Multicentric Castleman Disease: A Consensus-based Position Paper From an ad hoc Expert Panel
title_full Unmet Clinical Needs in the Management of Idiopathic Multicentric Castleman Disease: A Consensus-based Position Paper From an ad hoc Expert Panel
title_fullStr Unmet Clinical Needs in the Management of Idiopathic Multicentric Castleman Disease: A Consensus-based Position Paper From an ad hoc Expert Panel
title_full_unstemmed Unmet Clinical Needs in the Management of Idiopathic Multicentric Castleman Disease: A Consensus-based Position Paper From an ad hoc Expert Panel
title_short Unmet Clinical Needs in the Management of Idiopathic Multicentric Castleman Disease: A Consensus-based Position Paper From an ad hoc Expert Panel
title_sort unmet clinical needs in the management of idiopathic multicentric castleman disease: a consensus-based position paper from an ad hoc expert panel
topic Guideline Article – Consensus based
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10208718/
https://www.ncbi.nlm.nih.gov/pubmed/37234822
http://dx.doi.org/10.1097/HS9.0000000000000891
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