Regular prophylaxis with exogenous Factor VIII (FVIII) is recommended for individuals with severe haemophilia A (HA), but standardised data on bleeding, FVIII use, and quality of life (QoL) for this population in routine clinical practice are scarce. In a prospective, multinational, noninterventional study, male participants age ≥18 years with severe HA (FVIII ≤1 IU/dL) receiving prophylactic FVIII were followed for at least 6 months. Bleeding and FVIII use were reported weekly. Annualised bleeding rate (ABR) and FVIII infusion rate were calculated for participants with ≥6 months follow-up and combined with retrospective data from 6 months prior to baseline. Adverse events (AEs) were collected monthly. Participants completed the haemophilia-specific health-related QoL questionnaire for adults (Haemo-QoL-A) at baseline. This abstract presents a sub analysis of the study's Brazilian population. For all 54 Brazilian participants, median (range) age was 27 (18–47) years. For the 41 (76%) Brazilian participants with ≥6 months follow-up, mean (standard deviation [SD]; median) ABR for treated bleeds was 2.42 (4.05; 0.80) bleeds per year and mean (SD; median) annualised FVIII infusion rate was 167.87 (60.22; 155.17) infusions per year. Mean (SD; median) ABRs of treated traumatic, spontaneous, and joint bleeds were 0.90 (1.6; 0.0), 1.52 (3.3; 0.0), and 1.91 (3.29; 0.0) bleeds per year, respectively. Median (range) percentage of follow-up time adherent with prescribed prophylaxis frequency in Brazilian participants was 94.7%(49%-100%). Mean (SD) Haemo-QoL-A total transformed score (0–100) score was 70.3 (15.1) The lowest mean (SD) domain scores (representing lower QoL) in all Brazilian participants were for treatment concern 45.7 (30.85), physical functioning 66.9 (21.33), and consequences of bleeding 68.1 (22.92). AEs were reported in 22 (40.7%) participants, with the most common AEs being haemophilic arthropathy, nasopharyngitis, and synovitis, each reported in 2 (3.7%) Brazilian participants. Two serious AEs (oesophageal haemorrhage and haematoma) were reported, each by single participant (1.9%). Despite high prophylaxis adherence, participants reported breakthrough spontaneous and joint bleeding and impaired physical functioning. Additional haemostatic options for severe HA are needed.
O fator de impacto mede o número médio de citações recebidas em um ano por trabalhos publicados na revista durante os dois anos anteriores.
© Clarivate Analytics, Journal Citation Reports 2025
O CiteScore mede as citações médias recebidas por documento publicado. Mais informação
Ver maisSJR é uma métrica de prestígio baseada na idéia de que todas as citações não são iguais. SJR utiliza um algoritmo similar ao page rank do Google; é uma medida quantitativa e qualitativa ao impacto de uma publicação.
Ver maisSNIP permite comparar o impacto de revistas de diferentes campos temáticos, corrigindo as diferenças na probabilidade de ser citado que existe entre revistas de distintas matérias.
Ver mais




