ArticleHaematologica2016
Clinical, instrumental, serological and histological findings suggest that hemophilia B may be less severe than hemophilia A.
Article in Haematologica, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 64 citations in OpenAlex.
- Comparison of Total Joint Replacement Rate Between Patients With Hemophilia A and Patients With Hemophilia B: A Population-Based and Retrospective Cohort Study.Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis · 2018Trial
- Association of musculoskeletal impairments and balance with quality of life in adults with severe hemophilia A: a cross-sectional study.BMC musculoskeletal disorders · 2026Observational
- The relationship between joint health, kinesiophobia, physical activity level, and functional capacity in hemophilia patients: a case-control study.Acta orthopaedica et traumatologica turcica · 2026Article
- Benchmarking prophylaxis with factor concentrates: reference data on annualized bleeding rates in children with severe hemophilia.Haematologica · 2026Article
- Intracranial hemorrhage before start of prophylaxis in children with hemophilia: incidence, timing, and potential for prevention.Haematologica · 2025Observational
- Cellular stress and coagulation factor production: when more is not necessarily better.Journal of thrombosis and haemostasis : JTH · 2023Review
- Managing Relevant Clinical Conditions of Hemophilia A/B Patients.Hematology reports · 2023Article
- Ferroptosis: a new target for iron overload-induced hemophilic arthropathy synovitis.Annals of hematology · 2023Article
- The Profile of Markers of Bone Turnover, Inflammation and Extracellular Neutrophil Traps on Bone Mass in Haemophilia and the Development of Haemophilic Arthropathy.Journal of clinical medicine · 2022Article
- Pain in Hemophilia: Unexplored Role of Oxidative Stress.Antioxidants (Basel, Switzerland) · 2022Review
- Recurrent bleeding after rubber band ligation diagnosed as mild hemophilia B: a case report and literature review.BMC surgery · 2022Review
- FVIII regulates the molecular profile of endothelial cells: functional impact on the blood barrier and macrophage behavior.Cellular and molecular life sciences : CMLS · 2022Article
- Observational
- Recent Advances in the Treatment of Hemophilia: A Review.Biologics : targets & therapy · 2021Review
- Advances in gene therapy for hemophilia.Journal of biosciences · 2020Review
- Hemophilia A and B: molecular and clinical similarities and differences.Haematologica · 2019Review
- TNF-α/TNF-R System May Represent a Crucial Mediator of Proliferative Synovitis in Hemophilia A.Journal of clinical medicine · 2019Article
- Article
- Haemophilia B is clinically less severe than haemophilia A: further evidence.Blood transfusion = Trasfusione del sangue · 2018Article
- Pathophysiology of Hemophilic Arthropathy.Journal of clinical medicine · 2017Review
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Authors and funding
10 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Recent evidence suggests that patients with severe hemophilia B may have a less severe disease compared to severe hemophilia A. To investigate clinical, radiological, laboratory and histological differences in the arthropathy of severe hemophilia A and hemophilia B, 70 patients with hemophilia A and 35 with hemophilia B with at least one joint bleeding were consecutively enrolled. Joint bleedings (<10, 10-50, >50), regimen of treatment (prophylaxis/on demand), World Federation of Hemophilia, Pettersson and ultrasound scores, serum soluble RANK ligand and osteoprotegerin were assessed in all patients. RANK, RANK ligand and osteoprotegerin expression was evaluated in synovial tissue from 18 hemophilia A and 4 hemophilia B patients. The percentage of patients with either 10-50 or more than 50 hemarthrosis was greater in hemophilia A than in hemophilia B (P<0.001 and P=0.03, respectively), while that with less than 10 hemarthrosis was higher in hemophilia B (P<0.0001). World Federation of Hemophilia (36.6 vs. 20.2; P<0.0001) and ultrasound (10.9 vs. 4.3; P<0.0001) score mean values were significantly higher in hemophilia A patients. Serum osteoprotegerin and soluble RANK ligand were decreased in hemophilia A versus hemophilia B (P<0.0001 and P=0.006, respectively). Osteoprotegerin expression was markedly reduced in synovial tissue from hemophilia A patients. In conclusion, the reduced number of hemarthrosis, the lower World Federation of Hemophilia and ultrasound scores, and higher osteoprotegerin expression in serum and synovial tissue in hemophilia B suggest that hemophilia B is a less severe disease than hemophilia A. Osteoprotegerin reduction seems to play a pivotal role in the progression of arthropathy in hemophilia A.
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