Successful treatment of refractory hyperferritinemic syndromes with canakinumab: a report of two cases

BACKGROUND: Hyperferritinemic syndromes are systemic inflammatory disorders characterized by a dysfunctional immune response, which leads to excessive activation of the monocyte-macrophage system with hypercytokinemia and may pursue a rapidly fatal course. CASE PRESENTATION: We describe two patients...

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Autores principales: Papa, Riccardo, Natoli, Valentina, Caorsi, Roberta, Minoia, Francesca, Gattorno, Marco, Ravelli, Angelo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7353681/
https://www.ncbi.nlm.nih.gov/pubmed/32653009
http://dx.doi.org/10.1186/s12969-020-00450-9
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author Papa, Riccardo
Natoli, Valentina
Caorsi, Roberta
Minoia, Francesca
Gattorno, Marco
Ravelli, Angelo
author_facet Papa, Riccardo
Natoli, Valentina
Caorsi, Roberta
Minoia, Francesca
Gattorno, Marco
Ravelli, Angelo
author_sort Papa, Riccardo
collection PubMed
description BACKGROUND: Hyperferritinemic syndromes are systemic inflammatory disorders characterized by a dysfunctional immune response, which leads to excessive activation of the monocyte-macrophage system with hypercytokinemia and may pursue a rapidly fatal course. CASE PRESENTATION: We describe two patients of 11 and 9 years of age with hyperferritinemic syndromes, one with impending macrophage activation syndrome (MAS) and one with overt MAS, who were refractory or intolerant to conventional therapies, but improved dramatically with canakinumab. CONCLUSIONS: Our report indicates that canakinumab may be efficacious in the management of hyperferritinemic syndromes, including MAS.
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spelling pubmed-73536812020-07-14 Successful treatment of refractory hyperferritinemic syndromes with canakinumab: a report of two cases Papa, Riccardo Natoli, Valentina Caorsi, Roberta Minoia, Francesca Gattorno, Marco Ravelli, Angelo Pediatr Rheumatol Online J Case Report BACKGROUND: Hyperferritinemic syndromes are systemic inflammatory disorders characterized by a dysfunctional immune response, which leads to excessive activation of the monocyte-macrophage system with hypercytokinemia and may pursue a rapidly fatal course. CASE PRESENTATION: We describe two patients of 11 and 9 years of age with hyperferritinemic syndromes, one with impending macrophage activation syndrome (MAS) and one with overt MAS, who were refractory or intolerant to conventional therapies, but improved dramatically with canakinumab. CONCLUSIONS: Our report indicates that canakinumab may be efficacious in the management of hyperferritinemic syndromes, including MAS. BioMed Central 2020-07-11 /pmc/articles/PMC7353681/ /pubmed/32653009 http://dx.doi.org/10.1186/s12969-020-00450-9 Text en © The Author(s) 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Papa, Riccardo
Natoli, Valentina
Caorsi, Roberta
Minoia, Francesca
Gattorno, Marco
Ravelli, Angelo
Successful treatment of refractory hyperferritinemic syndromes with canakinumab: a report of two cases
title Successful treatment of refractory hyperferritinemic syndromes with canakinumab: a report of two cases
title_full Successful treatment of refractory hyperferritinemic syndromes with canakinumab: a report of two cases
title_fullStr Successful treatment of refractory hyperferritinemic syndromes with canakinumab: a report of two cases
title_full_unstemmed Successful treatment of refractory hyperferritinemic syndromes with canakinumab: a report of two cases
title_short Successful treatment of refractory hyperferritinemic syndromes with canakinumab: a report of two cases
title_sort successful treatment of refractory hyperferritinemic syndromes with canakinumab: a report of two cases
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7353681/
https://www.ncbi.nlm.nih.gov/pubmed/32653009
http://dx.doi.org/10.1186/s12969-020-00450-9
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