ArticlePediatric rheumatology online journal2019
Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome.
Article in Pediatric rheumatology online journal, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT05611710 (Anakinra for Dengue Patients With Hyperinflammation - a Randomized Double-blind Placebo-controlled Trial), which is not on this map. Cited by 52 papers, 2 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
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.
Anakinra for Dengue Patients With Hyperinflammation - a Randomized Double-blind Placebo-controlled Trial
Who cites it
52 citing papers in PubMed, 2 syntheses or guidelines pooled 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
- 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
- Advancements in Diagnosis and Care for Immune Dysregulation and Inborn Errors of Immunity in Pediatric Rheumatology: Our 50-Year Journey.Arthritis care & research · 2026Review
- Ruxolitinib prevents irreversible autoimmune endocrinopathies in APECED.Nature communications · 2026Article
- Identifying Hemophagocytic Lymphohistiocytosis and Describing Outcomes Using Computable Phenotypes: Retrospective Cohort Study.JMIR cancer · 2026Article
- CXCL9 as a novel prognostic marker to identify high-risk adults with hemophagocytic lymphohistiocytosis.Blood · 2026Article
- PRMT5 inhibition reduces hyperinflammation in a murine model of secondary hemophagocytic lymphohistiocytosis.Blood advances · 2025Article
- Article
- Haemophagocytic lymphohistiocytosis in critical care.BJA education · 2025Review
- Multidisciplinary approach to treating complex immune dysregulation disorders: an adaptive model for institutional implementation.Frontiers in immunology · 2025Review
- Treating and triggering hyperinflammation: tackling hemophagocytic lymphohistiocytosis and HLH-like syndromes in the pediatric cell therapy and critical care setting.Frontiers in oncology · 2025Review
- Development of Preliminary Criteria of Macrophage Activation Syndrome in Multisystem Inflammatory Syndrome Associated with COVID-19 in Children.Biomedicines · 2024Article
- Dynamics of Biomarkers in COVID-19 Patients Treated with Anakinra.Biomedicines · 2024Article
- [Hemophagocytic lymphohistiocytosis and macrophage activation syndrome : A multidisciplinary challenge].Zeitschrift fur Rheumatologie · 2024Review
- Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin.Advances in rheumatology (London, England) · 2024Review
- Identification of miRNAs that target Fcγ receptor-mediated phagocytosis during macrophage activation syndrome.Frontiers in immunology · 2024Article
- Anakinra Removal by Continuous Renal Replacement Therapy: An Ex Vivo Analysis.Critical care explorations · 2023Article
- Clinical and laboratory features associated with macrophage activation syndrome in Still's disease: data from the international AIDA Network Still's Disease Registry.Internal and emergency medicine · 2023Article
- CAR T-Cells for the Treatment of B-Cell Acute Lymphoblastic Leukemia.Journal of clinical medicine · 2023Review
- Use of Intravenous Anakinra for Management of Pediatric Cytokine Storm Syndromes at an Academic Medical Center.Hospital pharmacy · 2023Article
Corrections and comments
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Authors and funding
26 authors.
Funding
Abstract
backgroundHemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) were historically thought to be distinct entities, often managed in isolation. In fact, these conditions are closely related. A collaborative approach, which incorporates expertise from subspecialties that previously treated HLH/MAS independently, is needed. We leveraged quality improvement (QI) techniques in the form of an Evidence-Based Guideline (EBG) to build consensus across disciplines on the diagnosis and treatment of HLH/MAS.
methodsA multidisciplinary work group was convened that met monthly to develop the HLH/MAS EBG. Literature review and expert opinion were used to develop a management strategy for HLH/MAS. The EBG was implemented, and quality metrics were selected to monitor outcomes.
resultsAn HLH/MAS clinical team was formed with representatives from subspecialties involved in the care of patients with HLH/MAS. Broad entry criteria for the HLH/MAS EBG were established and included fever and ferritin ≥500 ng/mL. The rheumatology team was identified as the "gate-keeper," charged with overseeing the diagnostic evaluation recommended in the EBG. First-line medications were recommended based on the acuity of illness and risk of concurrent infection. Quality metrics to be tracked prospectively based on time to initiation of treatment and clinical response were selected.
conclusionHLH/MAS are increasingly considered to be a spectrum of related conditions, and joint management across subspecialties could improve patient outcomes. Our experience in creating a multidisciplinary approach to HLH/MAS management can serve as a model for care at other institutions.
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Registered trials
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.