ArticleInternational journal of hematology2022
Anakinra for the treatment of adult secondary HLH: a retrospective experience.
Article in International journal of hematology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 28 citations in OpenAlex.
- Hemophagocytic lymphohistiocytosis in critically ill patients: A guide for clinicians.World journal of critical care medicine · 2026Review
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- Late-onset hemophagocytic lymphohistiocytosis associated with monoallelic STX11 mutation in an adult: a case report and rationale for early allogeneic hematopoietic cell transplantation.Annals of hematology · 2025Article
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- Fatal Cytokine Collision: HLH-AIHA in Advanced AIDS-Case Report and Literature Review.Reports (MDPI) · 2025Article
- Hemophagocytic Lymphohistiocytosis After Treatment With Checkpoint Inhibitor Therapy.Journal of medical cases · 2025Article
- Promising Targeted Therapies for Hemophagocytic Lymphohistiocytosis: A Translational Perspective Based on Immunopathology.Clinical reviews in allergy & immunology · 2025Review
- Emapalumab Treatment in Patients With Rheumatologic Disease-Associated Hemophagocytic Lymphohistiocytosis in the United States: A Retrospective Medical Chart Review Study.Arthritis & rheumatology (Hoboken, N.J.) · 2025Article
- Low-dose emapalumab treatment in refractory macrophage activation syndrome secondary to adult onset still's disease/systemic lupus erythematosus: insights from nine cases.Frontiers in immunology · 2025Article
- Macrophage activation syndrome in Sepsis: from pathogenesis to clinical management.Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2024Review
- Article
- Emapalumab as salvage therapy for adults with malignancy-associated hemophagocytic lymphohistiocytosis.Haematologica · 2024Article
- The NLRP3 inflammasome is essential for IL-18 production in a murine model of macrophage activation syndrome.Disease models & mechanisms · 2024Article
- The NLRP3 inflammasome is essential for IL-18 production in a murine model of macrophage activation syndrome.bioRxiv : the preprint server for biology · 2024Article
- Improved survival outcomes with anakinra over etoposide-based therapies for the management of adults with hemophagocytic lymphohistiocytosis: a retrospective multicenter research network study.Therapeutic advances in hematology · 2024Article
- IL-1 Family Blockade in Cytokine Storm Syndromes.Advances in experimental medicine and biology · 2024Review
- Immune Effector Cell-Associated HLH-like Syndrome: A Review of the Literature of an Increasingly Recognized Entity.Cancers · 2023Review
- Diagnosis and Management of Adult Malignancy-Associated Hemophagocytic Lymphohistiocytosis.Cancers · 2023Review
- Article
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Authors and funding
1 author at 1 institution in 1 country.
Funding
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Abstract
Anti-cytokine therapies have been gaining attention as a means of improving outcomes in adult secondary HLH (asHLH), which currently has poor outcomes when treated with standard etoposide-based therapies. Anakinra is an interleukin-1 antagonist that is increasingly being used in the management of asHLH. Here is described a multi-hospital series of 16 adult patients with secondary HLH treated with anakinra. Provoking factors of secondary HLH included hematologic malignancy (n = 7, 44%), bacterial infection (n = 7, 44%), viral infection (n = 5, 31%), rheumatologic disorder (n = 4, 25%), and unknown (n = 1, 6%). Five patients remained alive at time of last follow-up (OS = 31%). Median OS was 1.7 months from initiation of anakinra (range 0.2-59). OS among patients with rheumatologic causes of secondary HLH was 75%, whereas only 17% of patients with other provoking factors survived (p = 0.0293). Anakinra was well tolerated, with only 1 patient experiencing associated toxicity (grade 3 liver injury). Anakinra may be useful in the management of asHLH provoked by rheumatologic conditions, although its benefit in asHLH provoked by other factors may be limited.
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