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Anakinra for the Treatment of Hemophagocytic Lymphohistiocytosis: 21 Cases

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening cytokine storm syndrome. There are no definitive guidelines for the management of secondary HLH (sHLH). A recent report by a National Health Service (NHS) clinical panel has recommended anakinra as a treatment option. We aimed to analyse...

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Autores principales: Baverez, Clara, Grall, Maximilien, Gerfaud-Valentin, Mathieu, De Gail, Sarah, Belot, Alexandre, Perpoint, Thomas, Weber, Emmanuelle, Reynaud, Quitterie, Sève, Pascal, Jamilloux, Yvan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9572845/
https://www.ncbi.nlm.nih.gov/pubmed/36233667
http://dx.doi.org/10.3390/jcm11195799
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author Baverez, Clara
Grall, Maximilien
Gerfaud-Valentin, Mathieu
De Gail, Sarah
Belot, Alexandre
Perpoint, Thomas
Weber, Emmanuelle
Reynaud, Quitterie
Sève, Pascal
Jamilloux, Yvan
author_facet Baverez, Clara
Grall, Maximilien
Gerfaud-Valentin, Mathieu
De Gail, Sarah
Belot, Alexandre
Perpoint, Thomas
Weber, Emmanuelle
Reynaud, Quitterie
Sève, Pascal
Jamilloux, Yvan
author_sort Baverez, Clara
collection PubMed
description Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening cytokine storm syndrome. There are no definitive guidelines for the management of secondary HLH (sHLH). A recent report by a National Health Service (NHS) clinical panel has recommended anakinra as a treatment option. We aimed to analyse the efficacy and safety of anakinra for the treatment of all-cause sHLH. We conducted a multicentric retrospective study in two French University hospitals and included all patients who had a diagnosis of sHLH and who received anakinra. Among 21 patients (median age, 45 years), 13 were men. Anakinra was used as first-line therapy in 10 patients, and as monotherapy in 5 patients. We found that anakinra was effective in 19/21 patients (90.5%), with fever resolution in 19 patients (90.5%) within a median of 1.0 day (1, 2). At the Day 7 assessment, the mean CRP concentration decreased significantly (p < 0.001), as did the mean ferritin (p = 0.011). Anakinra was generally safe and well tolerated and was discontinued for side effects in only three patients (14.3%). Anakinra is an efficient and safe treatment to control sHLH of various causes. These data, together with the recent report of the NHS panel, call for the rapid conduct of prospective randomized clinical trials.
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spelling pubmed-95728452022-10-17 Anakinra for the Treatment of Hemophagocytic Lymphohistiocytosis: 21 Cases Baverez, Clara Grall, Maximilien Gerfaud-Valentin, Mathieu De Gail, Sarah Belot, Alexandre Perpoint, Thomas Weber, Emmanuelle Reynaud, Quitterie Sève, Pascal Jamilloux, Yvan J Clin Med Article Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening cytokine storm syndrome. There are no definitive guidelines for the management of secondary HLH (sHLH). A recent report by a National Health Service (NHS) clinical panel has recommended anakinra as a treatment option. We aimed to analyse the efficacy and safety of anakinra for the treatment of all-cause sHLH. We conducted a multicentric retrospective study in two French University hospitals and included all patients who had a diagnosis of sHLH and who received anakinra. Among 21 patients (median age, 45 years), 13 were men. Anakinra was used as first-line therapy in 10 patients, and as monotherapy in 5 patients. We found that anakinra was effective in 19/21 patients (90.5%), with fever resolution in 19 patients (90.5%) within a median of 1.0 day (1, 2). At the Day 7 assessment, the mean CRP concentration decreased significantly (p < 0.001), as did the mean ferritin (p = 0.011). Anakinra was generally safe and well tolerated and was discontinued for side effects in only three patients (14.3%). Anakinra is an efficient and safe treatment to control sHLH of various causes. These data, together with the recent report of the NHS panel, call for the rapid conduct of prospective randomized clinical trials. MDPI 2022-09-30 /pmc/articles/PMC9572845/ /pubmed/36233667 http://dx.doi.org/10.3390/jcm11195799 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Baverez, Clara
Grall, Maximilien
Gerfaud-Valentin, Mathieu
De Gail, Sarah
Belot, Alexandre
Perpoint, Thomas
Weber, Emmanuelle
Reynaud, Quitterie
Sève, Pascal
Jamilloux, Yvan
Anakinra for the Treatment of Hemophagocytic Lymphohistiocytosis: 21 Cases
title Anakinra for the Treatment of Hemophagocytic Lymphohistiocytosis: 21 Cases
title_full Anakinra for the Treatment of Hemophagocytic Lymphohistiocytosis: 21 Cases
title_fullStr Anakinra for the Treatment of Hemophagocytic Lymphohistiocytosis: 21 Cases
title_full_unstemmed Anakinra for the Treatment of Hemophagocytic Lymphohistiocytosis: 21 Cases
title_short Anakinra for the Treatment of Hemophagocytic Lymphohistiocytosis: 21 Cases
title_sort anakinra for the treatment of hemophagocytic lymphohistiocytosis: 21 cases
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9572845/
https://www.ncbi.nlm.nih.gov/pubmed/36233667
http://dx.doi.org/10.3390/jcm11195799
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