{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T21:01:15Z","timestamp":1790283675767,"version":"4.1.0"},"reference-count":14,"publisher":"MDPI AG","issue":"19","license":[{"start":{"date-parts":[[2022,9,30]],"date-time":"2022-09-30T00:00:00Z","timestamp":1664496000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>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 &lt; 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.<\/jats:p>","DOI":"10.3390\/jcm11195799","type":"journal-article","created":{"date-parts":[[2022,10,8]],"date-time":"2022-10-08T23:39:31Z","timestamp":1665272371000},"page":"5799","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":42,"title":["Anakinra for the Treatment of Hemophagocytic Lymphohistiocytosis: 21 Cases"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2342-2850","authenticated-orcid":false,"given":"Clara","family":"Baverez","sequence":"first","affiliation":[{"name":"Service de M\u00e9decine Interne, H\u00f4pital de la Croix Rousse, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69008 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2962-9828","authenticated-orcid":false,"given":"Maximilien","family":"Grall","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Intensive et R\u00e9animation, H\u00f4pital Hospitalo\u2014Universitaire de Rouen, Universit\u00e9 de Rouen, 76000 Rouen, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4245-8039","authenticated-orcid":false,"given":"Mathieu","family":"Gerfaud-Valentin","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, H\u00f4pital de la Croix Rousse, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69008 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8082-6264","authenticated-orcid":false,"given":"Sarah","family":"De Gail","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, H\u00f4pital Hospitalo\u2014Universitaire de Rouen, Universit\u00e9 de Rouen, 76000 Rouen, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4902-5332","authenticated-orcid":false,"given":"Alexandre","family":"Belot","sequence":"additional","affiliation":[{"name":"Service de Rhumato-N\u00e9phro-Dermatologie P\u00e9diatrique, H\u00f4pital Femme M\u00e8re Enfants, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69008 Lyon, France"},{"name":"Lyon Immunopathology FEderation (LIFE), Universit\u00e9 Claude Bernard-Lyon 1, 69100 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Perpoint","sequence":"additional","affiliation":[{"name":"Service de Maladies Infectieuses et Tropicales, H\u00f4pital de la Croix Rousse, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69008 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuelle","family":"Weber","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, H\u00f4pital de la Croix Rousse, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69008 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Quitterie","family":"Reynaud","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69008 Lyon, France"},{"name":"Research on Healthcare Performance (RESHAPE), INSERM U1290, University Claude Bernard Lyon 1, 69100 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3070-3842","authenticated-orcid":false,"given":"Pascal","family":"S\u00e8ve","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, H\u00f4pital de la Croix Rousse, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69008 Lyon, France"},{"name":"Research on Healthcare Performance (RESHAPE), INSERM U1290, University Claude Bernard Lyon 1, 69100 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5249-3650","authenticated-orcid":false,"given":"Yvan","family":"Jamilloux","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, H\u00f4pital de la Croix Rousse, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69008 Lyon, France"},{"name":"Lyon Immunopathology FEderation (LIFE), Universit\u00e9 Claude Bernard-Lyon 1, 69100 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,9,30]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1016\/j.blre.2016.05.001","article-title":"Hemophagocytic syndromes (HPSs) including hemophagocytic 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Rev. Rheumatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1002\/pbc.21039","article-title":"HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis","volume":"48","author":"Henter","year":"2007","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1093\/rheumatology\/key006","article-title":"Macrophage activation syndrome in adults: Recent advances in pathophysiology, diagnosis and treatment","volume":"58","author":"Carter","year":"2019","journal-title":"Rheumatology"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1177\/1751143719893865","article-title":"Haemophagocytic lymphohistiocytosis in adult critical care","volume":"21","author":"Bauchmuller","year":"2020","journal-title":"J. Intensive Care Soc."},{"key":"ref_6","unstructured":"NHS England (2022, February 04). Anakinra for Haemophagocytic Lymphohistiocytosis for Adults and Children in All Ages n.d. Available online: https:\/\/www.england.nhs.uk\/publication\/anakinra-for-haemophagocytic-lymphohistiocytosis-for-adults-and-children-in-all-ages\/."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1002\/art.41103","article-title":"Benefit of Anakinra in Treating Pediatric Secondary Hemophagocytic Lymphohistiocytosis","volume":"72","author":"Eloseily","year":"2020","journal-title":"Arthritis Rheumatol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"e185","DOI":"10.1097\/PCC.0000000000001827","article-title":"Outcomes Analysis of Children Diagnosed with Hemophagocytic Lymphohistiocytosis in the PICU","volume":"20","author":"Gregory","year":"2019","journal-title":"Pediatr. Crit. 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