{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T23:52:06Z","timestamp":1788306726651,"version":"build-2803163510"},"reference-count":22,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2020,7,29]],"date-time":"2020-07-29T00:00:00Z","timestamp":1595980800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2020,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Hemophagocytic lymphohistiocytosis (HLH) can be familial or secondary, which is often triggered by infection or malignancy. HLH therapy includes dexamethasone and etoposide. However, therapy is associated with significant morbidity and mortality. Anakinra, a recombinant interleukin\u20101 receptor antagonist, has been reported to treat macrophage activation syndrome (MAS), rheumatic sHLH. We report our experience with anakinra to treat patients with nonrheumatic secondary HLH (sHLH).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Procedure<\/jats:title>\n                    <jats:p>\n                      Six children were diagnosed with HLH from December 2014 to August 2016 and were treated with subcutaneous anakinra (6\u201010\u00a0mg\/kg\/day divided over four doses) with or without dexamethasone (10\u00a0mg\/m\n                      <jats:sup>2<\/jats:sup>\n                      \/day). Therapy was either escalated or weaned based on clinical and laboratory response.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Five of six patients were treated with anakinra and dexamethasone, and one with anakinra alone due to active cytomegalovirus (CMV) pneumonitis. The median age of diagnosis was 1.8\u00a0years (range 0.8\u201014.9\u00a0years). No pathogenic mutations associated with HLH were identified, but three of six possessed genetic variants of unknown significance. Infectious triggers were identified for four patients and two patients had malignancies. The average treatment duration was 8\u00a0weeks with 3.5\u20105.5\u00a0years of follow up. No patient needed escalation of therapy to include etoposide. All patients achieved remission. Anakinra was well tolerated without significant adverse effects.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Initial treatment with anakinra (with or without dexamethasone) is a feasible treatment alternative for patients with secondary HLH and may allow for avoidance of etoposide. We recommend early initiation of anakinra when HLH is suspected. A broader investigation of the use of anakinra as a first\u2010line agent for HLH is ongoing.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/pbc.28581","type":"journal-article","created":{"date-parts":[[2020,7,29]],"date-time":"2020-07-29T10:33:13Z","timestamp":1596018793000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":85,"title":["The use of anakinra in the treatment of secondary hemophagocytic lymphohistiocytosis"],"prefix":"10.1002","volume":"67","author":[{"given":"Sakshi","family":"Bami","sequence":"first","affiliation":[{"name":"Hospitalist Medicine Program St Jude Children's Research Hospital  Memphis Tennessee"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anshul","family":"Vagrecha","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Hematology\/Oncology and Cellular Therapy Cohen Children's Medical Center  New Hyde Park New York"},{"name":"Zucker School of Medicine at Hofstra\/Northwell  Hempstead New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3763-9476","authenticated-orcid":false,"given":"Danielle","family":"Soberman","sequence":"additional","affiliation":[{"name":"Zucker School of Medicine at Hofstra\/Northwell  Hempstead New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5131-9861","authenticated-orcid":false,"given":"Mohamad","family":"Badawi","sequence":"additional","affiliation":[{"name":"Division of Pediatric Hematology\/Oncology West Virginia University  Charleston West Virginia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Cannone","sequence":"additional","affiliation":[{"name":"Division of Pediatric Hematology\/Oncology and Stem Cell Transplantation, Children's Hospital of Richmond Virginia Commonwealth University  Richmond Virginia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey M.","family":"Lipton","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Hematology\/Oncology and Cellular Therapy Cohen Children's Medical Center  New Hyde Park New York"},{"name":"Zucker School of Medicine at Hofstra\/Northwell  Hempstead New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Randy Q.","family":"Cron","sequence":"additional","affiliation":[{"name":"Division of Pediatric Rheumatology, Children's Hospital of Alabama University of Alabama  Birmingham Alabama"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9285-2248","authenticated-orcid":false,"given":"Carolyn Fein","family":"Levy","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Hematology\/Oncology and Cellular Therapy Cohen Children's Medical Center  New Hyde Park New York"},{"name":"Zucker School of Medicine at Hofstra\/Northwell  Hempstead New 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