Thanks to Elaine Benoit, Precision BioLogic Inc., for the heads-up on this newly released hemophilia study revealing a higher prevalence than previously reported. Also, the life expectancy disadvantage data are striking.
Researchers from McMaster University, North Carolina State University, Aix-Marseille University, and the University of Sheffield joined efforts to respond to a call to action of the World Federation of Hemophilia (WFH) to provide precise estimates of the prevalence and prevalence at birth of hemophilia and the associated life expectancy disadvantage for male patients with hemophilia A or B, which is critical information needed to plan for the improvement of hemophilia care worldwide. The authors conducted a meta-analysis of registry data in countries with comprehensive registries (Australia, Canada, France, Italy, New Zealand, and the United Kingdom) and found that prevalence of hemophilia is higher than previously estimated. The authors report a prevalence, per 100,000 males, of 17 for all severities of hemophilia A, 6 for severe hemophilia A, 4 for all severities of hemophilia B, and 1 for severe hemophilia B. For the first time, the prevalence at birth of hemophilia, defined as the number of people born with hemophilia per 100,000 male births, was estimated. The prevalence at birth per 100,000 males is 25 for all severities of hemophilia A, 10 for severe hemophilia A, 5 for all severities of hemophilia B and 2 for severe hemophilia B. This translates to approximately 1,125,000 males with hemophilia worldwide, of whom about 418,000 have severe hemophilia, the majority of whom are undiagnosed. The life expectancy disadvantage for people with hemophilia living in high-income countries is 30% for hemophilia A, 37% for severe hemophilia A, 24% for hemophilia B, and 27% for severe hemophilia B. The gap in life expectancy for patients with hemophilia is considerably worse in lower-income countries, where lack of treatment has affected the hemophilia population, and resulted in life expectancy disadvantages of approximately 64%, 77%, and 93% for countries with upper-middle, lower-middle, and low incomes, respectively, according to World Bank definitions.
Iorio A, Stonebraker JS, Chambost H, et al. Establishing the male prevalence and prevalence at birth of hemophilia. A meta-analytic approach using national registries. Ann Intern Med. 10 September 2019 [Epub ahead of print]. doi:10.7326/M19-1208.