ReviewFrontiers in immunology2021
Use of the JAK Inhibitor Ruxolitinib in the Treatment of Hemophagocytic Lymphohistiocytosis.
Review in Frontiers in immunology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 112 papers, 5 of them syntheses that pooled it.
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Who cites it
112 citing papers in PubMed, 5 syntheses or guidelines pooled it, 164 citations in OpenAlex.
- EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease.Annals of the rheumatic diseases · 2024Guideline
- The 2022 EULAR/ACR Points to Consider at the Early Stages of Diagnosis and Management of Suspected Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS).Arthritis & rheumatology (Hoboken, N.J.) · 2023Pooled it
- The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS).Annals of the rheumatic diseases · 2023Pooled it
- JAK inhibitors and COVID-19.Journal for immunotherapy of cancer · 2022Pooled it
- COVID-19-Associated acute respiratory distress syndrome (CARDS): Mechanistic insights on therapeutic intervention and emerging trends.International immunopharmacology · 2021Pooled it
- Dose-escalating ruxolitinib for refractory hemophagocytic lymphohistiocytosis.Frontiers in immunology · 2023Trial
- Emapalumab plus conventional therapy with or without ruxolitinib for pediatric hemophagocytic lymphohistiocytosis: a single center retrospective study.Immunologic research · 2026Article
- Article
- Origin, diagnosis and treatment of periocular Histiocytic disorders- state-of-the-art review.Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie · 2026Review
- Chimeric antigen receptor T-cell therapies related to immune effector cell-associated hemophagocytic lymphohistiocytosis-like syndrome: Diagnosis, high-risk factors, and management.Chinese medical journal · 2026Review
- Ruxolitinib Penetrates Blood Brain Barrier and Reduces the Cytokine Storm in Patients With Haemophagocytic Lymphohistiocytosis.Journal of cellular and molecular medicine · 2026Article
- Chronic granulomatous disease with suspected secondary hemophagocytic lymphohistiocytosis: a case report and hypothesis-generating rationale for neuroimmune modulation.Critical care (London, England) · 2026Review
- Clinical experience with emapalumab treatment in a group of children with primary hemophagocytic lymphohistiocytosis.Journal of human immunity · 2026Article
- 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
- Peritoneal effusion as a marker of disease severity in secondary hemophagocytic lymphohistiocytosis: Clinical and immunological insights.SAGE open medicine · 2026Article
- SAVI: molecular mechanisms, clinical spectrum and precision medicine approaches beyond type-I IFN.Frontiers in immunology · 2026Review
- Case Report: Ruxolitinib for systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome: two pediatric cases and literature review.Frontiers in pediatrics · 2026Article
- Natural Killer/T-Cell Lymphoma-Associated Hemophagocytic lymphohistiocytosis-a Rare and Dangerous Disease.Blood and lymphatic cancer : targets and therapy · 2026Review
- Malignancy-Associated Hemophagocytic Lymphohistiocytosis: An Experience of 15 Years in Polish Pediatric Hematology Centers.Journal of blood medicine · 2026Article
- Childhood malignancy-associated hemophagocytic lymphohistiocytosis: a retrospective, single-center study of 44 patients.Frontiers in immunology · 2026Article
52 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome driven by overactive T cells and macrophages that abundantly secrete numerous pro-inflammatory cytokines, including interferon (IFN)-gamma, interleukin (IL)-1-beta, IL-2, IL-6, IL-10, IL-18, and tumor necrosis factor (TNF). The release of these and other cytokines underlies many of the clinical and pathologic manifestations of HLH, which if left untreated, can lead to multi-organ failure and death. The advent of etoposide-based regimens, such as the Histiocyte Society HLH-94 and HLH-2004 protocols, has substantially decreased the mortality associated with HLH. Nevertheless, the 5-year survival remains low at ~60%. To improve upon these results, studies have focused on the use of novel cytokine-directed therapies to dampen inflammation in HLH. Among the agents being tested is ruxolitinib, a potent inhibitor of the Janus Kinase (JAK) and Signal Transducer and Activation of Transcription (STAT) pathway, which functions downstream of many HLH-associated cytokines. Here, we review the basic biology of HLH, including the role of cytokines in disease pathogenesis, and discuss the use of ruxolitinib in the treatment of HLH.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.