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In a normal population, VWF plasma levels ( VWF:Ag) and VWF activity ( VWF: RCo) increase by approximately 0.17 and 0.15 IU mL−1 per decade, but the influence of age is unknown in patients with type 1 von Willebrand disease ( VWD). In a retrospective cohort study, the medical records of 31 type 1 VWD patients over the age of 30, who had been followed for ≥5 years, were reviewed for baseline clinical data and previously performed VWF:Ag, VWF: RCo and factor VIII levels ( FVIII:C). VWF multimer analysis was normal in 28/31 cases performed. Mean age at diagnosis was 33 (range 16-60 years), and duration of follow-up ranged from 5 to 26 years (mean 11 years). Patients had 2-10 time points of VWD testing (mean of 5.2). The mean VWF:Ag, VWF: RCo and FVIII:C at time of diagnosis were 0.44 IU mL−1 0.34 IU mL−1 and 0.75 IU mL−1. At last follow-up, the mean VWF:Ag, VWF: RCo and FVIII:C were significantly increased to 0.71 IU L−1, 0.56 IU mL−1 and 0.90 IU mL−1 ( P ≤ 0.001, <0.001, and 0.0081 respectively). Here 18/31 patients had VWF:Ag, VWF: RCo and FVIII: C levels that increased into the normal range. The rate of change in VWF:Ag, VWF: RCo and FVIII was 0.30 IU mL−1 (0.21-0.39, CI 95%, P < 0.0001), 0.20 IU mL−1 per decade (0.13-0.27, CI 95% , P = 0.0001) and 0.20 IU mL−1 (0.11-0.29, CI 95% , P = 0.0011). Patients with type 1 VWD experience age-related increases to VWF:Ag and VWF: RCo which can result in normalization of VWF levels. Further studies are required to determine if the bleeding phenotype resolves with the increases in VWF:Ag and VWF: RCo levels. [ABSTRACT FROM AUTHOR]

The article discussed a study conducted to investigate the utility of the Self-Bleeding Assessment Tools (BAT) in carriers and to evaluate correlations between bleeding score, FVIII/FIX level and quality of life (QoL). The study results found significant negative correlation between Self-BAT bleeding score (BSs) and QoL. It is noted that the study stresses the need to find determinants of bleeding in haemophilia carriers, and factors that contribute to their overall perception of health.