ArticlePediatric rheumatology online journal2021
Ruxolitinib treatment permits lower cumulative glucocorticoid dosing in children with secondary hemophagocytic lymphohistiocytosis.
Article in Pediatric rheumatology online journal, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers, 1 of them a synthesis that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
11 citing papers in PubMed, 1 synthesis or guideline pooled it, 14 citations in OpenAlex.
- Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project.Rheumatology (Oxford, England) · 2025Pooled it
- Accelerated response and reduced chemotherapy with ruxolitinib-based regimen therapy in pediatric hemophagocytic lymphohistiocytosis: a retrospective comparison with HLH-94 regimen.Frontiers in immunology · 2026Article
- Ruxolitinib for rheumatic disease-associated macrophage activation syndrome in children: a retrospective cohort study.Frontiers in immunology · 2026Article
- Article
- Macrophage Activation Syndrome in Kawasaki Disease: Insights from a Systematic Literature Review on Diagnosis, Clinical Features, and Treatment.Children (Basel, Switzerland) · 2025Review
- Efficacy of Ruxolitinib in Severe Pediatric Intra-Alveolar Hemorrhages Unrelated to COPA Mutations.Pediatric pulmonology · 2025Article
- Article
- JAK Inhibitors in Cytokine Storm Syndromes.Advances in experimental medicine and biology · 2024Review
- Cellular and transcriptional impacts of Janus kinase and/or IFN-gamma inhibition in a mouse model of primary hemophagocytic lymphohistiocytosis.Frontiers in immunology · 2023Article
- JAK inhibitors in systemic juvenile idiopathic arthritis.Frontiers in pediatrics · 2023Article
- Drugs for paediatric hyperinflammatory syndromes.Drugs in context · 2022Review
Corrections and comments
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
Abstract
backgroundThis study aimed to analyze the effects of ruxolitinib on children with secondary hemophagocytic lymphohistiocytosis (HLH).
methodsEleven pediatric patients diagnosed with HLH and treated with ruxolitinib (ruxolitinib group: group R) between November 2017 and August 2018 were retrospectively analyzed. Eleven age-matched pediatric patients with HLH undergoing conventional treatment (control group: group C) during the same period were also analyzed.
resultsIn group R, three patients who did not respond to methylprednisolone (MP) pulse and intravenous immunoglobulin (IVIG) therapies were treated with Ruxolitinib and their temperature decreased to normal levels. Four patients had normal temperature after conventional treatment (dexamethasone and etoposide, with or without cyclosporine A), but they had severe organ involvement, including obvious yellowing of the skin, increased liver enzyme levels and neuropsychiatric symptoms, and they were all ameliorated with ruxolitinib treatment. Four patients were relieved with ruxolitinib therapy alone. In group C, the body temperatures of eleven patients decreased to normal levels after conventional treatment. The body temperature of group R patients decreased to normal levels more rapidly than that of group C patients. The glucocorticoid dosage in group R was significantly lower than that in group C. Both groups were followed-up for 2-2.5 years. No obvious adverse drug reactions to ruxolitinib were observed during treatment and follow-up.
conclusionRuxolitinib might be an effective drug in controlling body temperature and reducing inflammation indicators. It might be a potential replacement for glucocorticoid therapy for HLH treatment in children, thereby reducing or avoiding glucocorticoid-related adverse reactions.
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