{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T22:25:28Z","timestamp":1783463128416,"version":"3.55.0"},"reference-count":27,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2026,5,14]],"date-time":"2026-05-14T00:00:00Z","timestamp":1778716800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,5,14]],"date-time":"2026-05-14T00:00:00Z","timestamp":1778716800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2026,8]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Immune effector cell\u2010associated hemophagocytic lymphohistiocytosis\u2010like syndrome (IEC\u2010HS) is a life\u2010threatening hyperinflammatory toxicity distinct from cytokine release syndrome (CRS) and neurotoxicity following chimeric antigen receptor T\u2010cell (CAR\u2010T) therapy. In a single\u2010institution retrospective cohort of pediatric and young adult patients with relapsed or refractory acute B\u2010lymphoblastic leukemia (B\u2010ALL) treated with tisagenlecleucel between November 2023 and April 2025, two of 12 patients (17%) developed IEC\u2010HS after CRS resolution, marked by recurrent fever, coagulopathy, hepatic dysfunction, cytopenias, and profound elevations in CXCL9, IL\u201018, and soluble C5b\u20109. Treatment with emapalumab led to rapid clinical and biomarker improvement. These findings support IEC\u2010HS as an interferon\u2010\u03b3\u2010driven syndrome and suggest targeted IFN\u2010\u03b3 blockade as a potentially effective therapeutic strategy.<\/jats:p>","DOI":"10.1002\/1545-5017.70406","type":"journal-article","created":{"date-parts":[[2026,5,15]],"date-time":"2026-05-15T05:21:26Z","timestamp":1778822486000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Emapalumab for Immune Effector Cell\u2010Associated Hemophagocytic Lymphohistiocytosis\u2010Like Syndrome Following CD19\u2010Directed CAR\u2010T in Two Patients With B\u2010ALL: Clinical and Biomarker Correlates"],"prefix":"10.1002","volume":"73","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6174-4319","authenticated-orcid":false,"given":"Thomas J.","family":"Galletta","sequence":"first","affiliation":[{"name":"Division of Oncology Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"},{"name":"Department of Pediatrics University of Cincinnati College of Medicine  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1934-2954","authenticated-orcid":false,"given":"Jeremy D.","family":"Rubinstein","sequence":"additional","affiliation":[{"name":"Division of Oncology Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"},{"name":"Department of Pediatrics University of Cincinnati College of Medicine  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4001-9203","authenticated-orcid":false,"given":"Christopher E.","family":"Dandoy","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Cincinnati College of Medicine  Cincinnati Ohio USA"},{"name":"Division of Bone Marrow Transplantation and Immune Deficiency Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruby","family":"Khoury","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Cincinnati College of Medicine  Cincinnati Ohio USA"},{"name":"Division of Bone Marrow Transplantation and Immune Deficiency Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela","family":"Faulhaber","sequence":"additional","affiliation":[{"name":"Division of Oncology Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5986-102X","authenticated-orcid":false,"given":"Christine L.","family":"Phillips","sequence":"additional","affiliation":[{"name":"Division of Oncology Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"},{"name":"Department of Pediatrics University of Cincinnati College of Medicine  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7050-6099","authenticated-orcid":false,"given":"Christa","family":"Krupski","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Cincinnati College of Medicine  Cincinnati Ohio USA"},{"name":"Division of Bone Marrow Transplantation and Immune Deficiency Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,5,14]]},"reference":[{"issue":"5","key":"e_1_2_9_2_1","first-page":"439","article-title":"Tisagenlecleucel in Children and Young Adults With B\u2010Cell Lymphoblastic Leukemia","volume":"378","author":"Maude S. 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