{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,9]],"date-time":"2026-06-09T11:58:42Z","timestamp":1781006322250,"version":"3.54.1"},"reference-count":19,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,6,9]],"date-time":"2026-06-09T00:00:00Z","timestamp":1780963200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>Hemophagocytic lymphohistiocytosis (HLH), a severe, life-threatening hyperinflammatory syndrome driven by dysregulated immune activation, is characterized by rapid clinical deterioration and poor outcomes that pose critical challenges for clinical management. Here, we report on a female patient aged 10 years and 3 months who was diagnosed with systemic lupus erythematosus (SLE)-associated hemophagocytic lymphohistiocytosis with central nervous system (CNS) involvement. Treatment with the conventional HLH-94\/2004 regimen failed to control disease progression. Subsequently, second-line salvage therapy, consisting of emapalumab in combination with teniposide and dexamethasone, was initiated to account for the CNS involvement. The patient achieved sustained, complete clinical response following use of this modified therapeutic approach. Our findings suggest that this combination regimen may offer a potentially feasible therapeutic alternative for managing patients with SLE-associated HLH accompanied by CNS involvement; however, given the single-case nature of this report, further validation in larger cohorts is needed.<\/jats:p>","DOI":"10.3389\/fimmu.2026.1796119","type":"journal-article","created":{"date-parts":[[2026,6,9]],"date-time":"2026-06-09T11:06:52Z","timestamp":1781003212000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Modified treatment protocol for pediatric systemic lupus erythematosus-associated hemophagocytic lymphohistiocytosis with central nervous system involvement: a case report"],"prefix":"10.3389","volume":"17","author":[{"given":"Mingda","family":"Tian","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Beijing United Family Women\u2019s and Children\u2019s Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yinzhu","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Beijing United Family Women\u2019s and Children\u2019s Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danyang","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Hematology, Beijing Lu He Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chunyi","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Beijing United Family Women\u2019s and Children\u2019s Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yang","family":"Zheng","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Beijing United Family Women\u2019s and Children\u2019s Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junjin","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Beijing United Family Women\u2019s and Children\u2019s Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hebing","family":"Zhou","sequence":"additional","affiliation":[{"name":"Department of Hematology, Beijing Lu He Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiayue","family":"Qin","sequence":"additional","affiliation":[{"name":"Department of Medical Affairs, Acornmed Biotechnology Co., Ltd.","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ming","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Beijing United Family Women\u2019s and Children\u2019s Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yan","family":"Yue","sequence":"additional","affiliation":[{"name":"Department of Hematology, Beijing Lu He Hospital","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,6,9]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1056\/nejmra2314005","article-title":"Hemophagocytic lymphohistiocytosis","volume":"392","author":"Henter","year":"2025","journal-title":"N Engl J Med"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1080\/17512433.2021.1901576","article-title":"Emapalumab for adult and pediatric patients with hemophagocytic lymphohistiocytosis","volume":"14","author":"Garonzi","year":"2021","journal-title":"Expert Rev Clin Pharmacol"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2019000936","article-title":"Pediatric hemophagocytic lymphohistiocytosis","volume":"135","author":"Canna","year":"2020","journal-title":"Blood"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1016\/s0099-5428(08)60671-1","article-title":"Etoposide and teniposide. Bioanalysis, metabolism and clinical pharmacokinetics","volume":"10","author":"Holthuis","year":"1988","journal-title":"Pharm Weekbl Sci"},{"key":"B5","doi-asserted-by":"publisher","first-page":"746","DOI":"10.1186\/s12885-023-11268-5","article-title":"First-line induction chemotherapy with high-dose methotrexate versus teniposide in patients with newly diagnosed primary central nervous system lymphoma: a retrospective, multicenter cohort study","volume":"23","author":"Zhong","year":"2023","journal-title":"BMC Cancer"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1056\/nejmoa1911326","article-title":"Emapalumab in children with primary hemophagocytic lymphohistiocytosis","volume":"382","author":"Locatelli","year":"2020","journal-title":"N Engl J Med"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1056\/nejmra2314005","article-title":"HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis","volume":"48","author":"Henter","year":"2007","journal-title":"Pediatr Blood Cancer"},{"key":"B8","doi-asserted-by":"publisher","first-page":"98","DOI":"10.1186\/s12969-024-01025-8","article-title":"Epstein-Barr virus encephalitis associated hemophagocytic lymphohistiocytosis in childhood-onset systemic lupus erythematosus: a case-based review","volume":"22","author":"Cheawcharnpraparn","year":"2024","journal-title":"Pediatr Rheumatol Online J"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20190147","article-title":"A novel disorder involving dyshematopoiesis, inflammation, and HLH due to aberrant CDC42 function","volume":"216","author":"Lam","year":"2019","journal-title":"J Exp Med"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2020.608492","article-title":"Novel therapeutic approaches to familial HLH (Emapalumab in FHL)","volume":"11","author":"Merli","year":"2020","journal-title":"Front Immunol"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1038\/s41409-024-02212-7","article-title":"Sequential haplo-identical conditioning transplant regimen for pediatric patients with relapsed or refractory hemophagocytic lymphohistiocytosis","volume":"59","author":"Yue","year":"2024","journal-title":"Bone Marrow Transplant"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-med-042921-112837","article-title":"Cytokine storm syndrome","volume":"74","author":"Cron","year":"2023","journal-title":"Annu Rev Med"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1007\/bf00684876","article-title":"Teniposide: overview of its therapeutic potential in adult cancers","author":"Muggia","year":"1994","journal-title":"Cancer Chemother Pharmacol"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1007\/s11060-018-2970-x","article-title":"Fotemustine, teniposide and dexamethasone versus high-dose methotrexate plus cytarabine in newly diagnosed primary CNS lymphoma: a randomised phase 2 trial","volume":"140","author":"Wu","year":"2018","journal-title":"J Neuro-Oncol"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.28900","article-title":"The role of interferon-gamma and its signaling pathway in pediatric hematological disorders","volume":"68","author":"Merli","year":"2021","journal-title":"Pediatr Blood Cancer"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2023012217","article-title":"Real-world treatment patterns and outcomes in patients with primary hemophagocytic lymphohistiocytosis treated with emapalumab","volume":"8","author":"Chandrakasan","year":"2024","journal-title":"Blood Adv"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.29026","article-title":"Combined use of emapalumab and ruxolitinib in a patient with refractory hemophagocytic lymphohistiocytosis was safe and effective","volume":"68","author":"Triebwasser","year":"2021","journal-title":"Pediatr Blood Cancer"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.4274\/balkanmedj.galenos.2022.2022-4-83","article-title":"Hemophagocytic lymphohistiocytosis","volume":"39","author":"Kacar","year":"2022","journal-title":"Balkan Med J"},{"key":"B19","doi-asserted-by":"publisher","first-page":"106","DOI":"10.1186\/s13045-024-01621-x","article-title":"Hemophagocytic lymphohistiocytosis: current treatment advances, emerging targeted therapy and underlying mechanisms","volume":"17","author":"Wu","year":"2024","journal-title":"J Hematol Oncol"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2026.1796119\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,9]],"date-time":"2026-06-09T11:06:53Z","timestamp":1781003213000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2026.1796119\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6,9]]},"references-count":19,"alternative-id":["10.3389\/fimmu.2026.1796119"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2026.1796119","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6,9]]},"article-number":"1796119"}}