ArticleBlood advances2022
The bleeding phenotype in people with nonsevere hemophilia.
Article in Blood advances, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 30 citations in OpenAlex.
- The effect of F8 missense variants on desmopressin response in people with nonsevere hemophilia A investigated using machine learning.Research and practice in thrombosis and haemostasis · 2026Article
- Characterization of a factor VIII/immunoglobulin heavy chain μ double-knockout mouse model of hemophilia A for long-term exposure to factor VIII proteins.Research and practice in thrombosis and haemostasis · 2026Article
- Relationship between Dose, Factor IX Activity Levels and Bleeding Probability for rIX-FP Prophylaxis in Hemophilia B: A Repeated Time-to-Event Analysis.Clinical pharmacology and therapeutics · 2025Article
- Global estimation of the bleeding episodes treatable with desmopressin in von Willebrand disease and hemophilia A.Haematologica · 2025Review
- Gene therapy for hemophilia - From basic science to first approvals of "one-and-done" therapies.Molecular therapy : the journal of the American Society of Gene Therapy · 2025Review
- Subjective Physical Performance and Its Determinants in Patients With Haemophilia.Haemophilia : the official journal of the World Federation of Hemophilia · 2025Article
- Mild or moderate hemophilia is not always a mild or moderate bleeding disorder: Back to the clinical phenotype.HemaSphere · 2025Article
- Nonsevere Hemophilia: The Need for a Renewed Focus and Improved Outcomes.Seminars in thrombosis and hemostasis · 2025Review
- Extending health equity to people with moderate and mild hemophilia A: revisiting the HAVEN 6 trial.Research and practice in thrombosis and haemostasis · 2025Article
- The role of public health in rare diseases: hemophilia as an example.Frontiers in public health · 2025Review
- Racial and Ethnic Differences in Distress, Depression, and Quality of Life in people with hemophilia.Journal of racial and ethnic health disparities · 2024Article
- The imperative to prevent joint bleeding in everyone living with hemophilia.Research and practice in thrombosis and haemostasis · 2024Article
- Patient perspective on living with mild hemophilia in Germany: results from a nationwide survey.Frontiers in medicine · 2024Article
- Minimum factor VIII levels to prevent joint bleeding in mild hemophilia A.Blood advances · 2023Article
- Mild and Moderate Hemophilia A: Neglected Conditions, Still with Unmet Needs.Journal of clinical medicine · 2023Article
- Bleeding phenotype in nonsevere hemophilia by International Society on Thrombosis and Haemostasis bleeding assessment tool, bleeding frequency, and the joint status.Research and practice in thrombosis and haemostasis · 2023Article
- Challenges in the Diagnosis and Management of Non-Severe Hemophilia.Journal of clinical medicine · 2022Article
- Delphi Consensus Recommendations for the Definition of a Severe Bleeding Phenotype and Initiation of Prophylaxis in Patients With Non-Severe Haemophilia.Haemophilia : the official journal of the World Federation of HemophiliaArticle
Corrections and comments
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Authors and funding
18 authors at 13 institutions in 4 countries.
Funding
Abstract
Detailed information on the onset, frequency, and severity of bleeding in nonsevere hemophilia is limited. We aimed to assess the bleeding phenotype of persons with nonsevere hemophilia and to analyze the association between baseline factor VIII/IX (FVIII/IX) levels and the joint bleeding rate. In the DYNAMO (Dynamic Interplay Between Bleeding Phenotype and Baseline Factor Level in Moderate and Mild Hemophilia A and B) study, an international multicenter cohort, we included males with nonsevere hemophilia (FVIII/IX, 0.02-0.35 IU/mL) aged 12 to 55 years. Information on age at first treated (joint) bleed, annual bleeding rates (ABRs), and annual joint bleeding rates (AJBRs) was collected from the medical files. The association between baseline FVIII/IX levels and the joint bleeding rate was assessed by using a frailty model for recurrent events. In total, 304 persons (70 with moderate hemophilia and 234 with mild hemophilia) were included. The median age was 38 years (interquartile range [IQR], 25-49 years), and the median baseline FVIII/IX level was 0.12 IU/mL (IQR, 0.05-0.21 IU/mL). In total, 245 (81%) persons had experienced at least 1 bleed, and 156 (51%) had experienced at least 1 joint bleed. The median age at first bleed and first joint bleed was 8 and 10 years, respectively. The median ABR and AJBR was 0.2 (IQR, 0.1-0.5) and 0.0 (IQR, 0.0-0.2). From baseline FVIII/IX levels 0.02 to 0.05 IU/mL to >0.25 IU/mL, the median ABR decreased from 0.6 (IQR, 0.2-1.4) to 0.1 (IQR, 0.0-0.2) and the AJBR from 0.2 (IQR, 0.0-0.4) to 0.0 (IQR, 0.0-0.0). Baseline FVIII/IX was inversely associated with the joint bleeding rate (P < .001). Low bleeding rates were observed in persons with nonsevere hemophilia. However, one-half of all adolescents and adults had experienced a joint bleed.
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