Cargando…
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...
Autores principales: | , , , , , , , , , , |
---|---|
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 |
_version_ | 1785046730357604352 |
---|---|
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. |
format | Online Article Text |
id | pubmed-10208718 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Lippincott Williams & Wilkins |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT zinzanipierluigi unmetclinicalneedsinthemanagementofidiopathicmulticentriccastlemandiseaseaconsensusbasedpositionpaperfromanadhocexpertpanel AT paullimarco unmetclinicalneedsinthemanagementofidiopathicmulticentriccastlemandiseaseaconsensusbasedpositionpaperfromanadhocexpertpanel AT arcainiluca unmetclinicalneedsinthemanagementofidiopathicmulticentriccastlemandiseaseaconsensusbasedpositionpaperfromanadhocexpertpanel AT dellatorreemanuel unmetclinicalneedsinthemanagementofidiopathicmulticentriccastlemandiseaseaconsensusbasedpositionpaperfromanadhocexpertpanel AT ferrerosimone unmetclinicalneedsinthemanagementofidiopathicmulticentriccastlemandiseaseaconsensusbasedpositionpaperfromanadhocexpertpanel AT figueraamalia unmetclinicalneedsinthemanagementofidiopathicmulticentriccastlemandiseaseaconsensusbasedpositionpaperfromanadhocexpertpanel AT frigeriferdinando unmetclinicalneedsinthemanagementofidiopathicmulticentriccastlemandiseaseaconsensusbasedpositionpaperfromanadhocexpertpanel AT martellimaurizio unmetclinicalneedsinthemanagementofidiopathicmulticentriccastlemandiseaseaconsensusbasedpositionpaperfromanadhocexpertpanel AT sabattinielena unmetclinicalneedsinthemanagementofidiopathicmulticentriccastlemandiseaseaconsensusbasedpositionpaperfromanadhocexpertpanel AT scarpariccardo unmetclinicalneedsinthemanagementofidiopathicmulticentriccastlemandiseaseaconsensusbasedpositionpaperfromanadhocexpertpanel AT barosigiovanni unmetclinicalneedsinthemanagementofidiopathicmulticentriccastlemandiseaseaconsensusbasedpositionpaperfromanadhocexpertpanel |