Trial reportPloS one2021
Coagulation disorders in patients with severe hemophagocytic lymphohistiocytosis.
Trial report in PloS one, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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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.
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Who cites it
22 citing papers in PubMed, 34 citations in OpenAlex.
- Coagulation dysfunction in children with secondary hemophagocytic lymphohistiocytosis: a comprehensive analysis.Annals of medicine · 2026Article
- Hemophagocytic lymphohistiocytosis in critically ill patients: A guide for clinicians.World journal of critical care medicine · 2026Review
- Diagnostic performance of HScore for macrophage activation syndrome complicating Still's disease in adults: a multicentre case-control study.Rheumatology (Oxford, England) · 2026Article
- Development and validation of machine learning models for early diagnosis of hemophagocytic lymphohistiocytosis in pediatric Epstein-Barr virus infection.BMC medical informatics and decision making · 2026Article
- Development and internal validation of a nomogram for predicting 30-day mortality in older patients with hemophagocytic lymphohistiocytosis.BMC geriatrics · 2026Article
- Integration of Boruta algorithm and latent class analysis for risk factors of 30-day mortality in pediatric hemophagocytic lymphohistiocytosis based on peripheral blood indicators.Frontiers in pediatrics · 2026Article
- Hemophagocytic Syndromes in Adults: Real-World Data on Mortality from a Tertiary Reference Center.Acta haematologica · 2026Article
- Influenza B-Triggered Secondary Hemophagocytic Lymphohistiocytosis in an Adult Male: A Diagnostic Challenge.Cureus · 2025Article
- Fatal Cytokine Collision: HLH-AIHA in Advanced AIDS-Case Report and Literature Review.Reports (MDPI) · 2025Article
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- Prognostic factors in patients with secondary hemophagocytic lymphohistioc ytosis in a Chinese cohort.Annals of hematology · 2024Observational
- Cytokine Storm and Sepsis-Induced Multiple Organ Dysfunction Syndrome.Advances in experimental medicine and biology · 2024Review
- Report of Two Contrasting Cases of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis: Comparison to Infectious Mononucleosis and Flow Cytometric Analysis of Bone Marrow.International medical case reports journal · 2024Article
- Hemophagocytic lymphohistiocytosis is associated with deficiency and closed conformation of ADAMTS-13.Research and practice in thrombosis and haemostasis · 2024Article
- Comparative study of biomarkers for the early identification of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in infectious mononucleosis.BMC infectious diseases · 2023Article
- The Crossroads of the Coagulation System and the Immune System: Interactions and Connections.International journal of molecular sciences · 2023Review
- Immune Effector Cell-Associated Hemophagocytic Lymphohistiocytosis-Like Syndrome.Transplantation and cellular therapy · 2023Article
- Analysis of Prognostic Risk Factors and Establishment of Prognostic Scoring System for Secondary Adult Hemophagocytic Syndrome.Current oncology (Toronto, Ont.) · 2022Article
- A Study on Early Death Prognosis Model in Adult Patients with Secondary Hemophagocytic Lymphohistiocytosis.Journal of healthcare engineering · 2022Article
Corrections and comments
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Authors and funding
16 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundCoagulation disorders are common in patients with hemophagocytic lymphohistiocytosis (HLH), associated with an increased risk of bleeding and death. We aim to investigate coagulation disorders and their outcome implications in critically ill patients with HLH.
methodsWe prospectively evaluated 47 critically ill patients with HLH (median age of 54 years [42-67]) between April 2015 and December 2018. Coagulation assessments were performed at day 1. Abnormal standard coagulation was defined as prothrombin time (PT) <50% and/or fibrinogen <2g/L. HLH aetiology was mostly ascribed to haematological malignancies (74% of patients).
resultsCoagulation disorders and severe bleeding events were frequent, occurring in 30 (64%) and 11 (23%) patients respectively. At day 1, median fibrinogen level was 2∙65g/L [1.61-5.66]. Fibrinolytic activity was high as suggested by increased median levels of D-dimers, fibrin monomers, PAI-1 (plasminogen activator inhibitor) and tPA (tissue plasminogen activator). Forty-one (91%) patients had a decreased ADAMTS13 activity (A Disintegrin-like And Metalloproteinase with ThromboSpondin type 1 repeats, member 13). By multivariable analysis, the occurrence of a severe bleeding (OR 3.215 [1.194-8.653], p = 0∙021) and SOFA score (Sepsis-Related Organ Failure Assessment) at day 1 (OR 1.305 per point [1.146-1.485], p<0∙001) were independently associated with hospital mortality. No early biological marker was associated with severe bleeding.
conclusionsHyperfibrinolysis may be the primary mechanism responsible for hypofibrinogenemia and may also participate in ADAMTS13 degradation. Targeting the plasmin system appears as a promising approach in severe HLH-related coagulation disorders.
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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.