The incidence and mortality rates of intracranial hemorrhage are higher in hemophilia patients than in the general population, according to the findings of a systematic review and meta-analysis published in the journal Blood.

“Our findings suggest that [intracranial hemorrhage] is an important problem in hemophilia that occurs among all ages, requiring adequate preventive strategies,” wrote Anne-Fleur Zwagemaker from Amsterdam University Medical Centers in the Netherlands, and the co-authors of the study.

It was already known that intracranial hemorrhage was relatively common among patients with hemophilia, but the precise incidence and mortality rates associated with it were not clear.

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Here, the researchers conducted a systematic literature review and meta-analysis. They identified 45 studies comprising 54,470 patients, 809,151 person-years, and 5326 live births of hemophilia patients.

They found that the incidence rate of intracranial hemorrhage was 2.3 per 1000 person-years and the rate of mortality was 0.8 per 1000 person-years for people with hemophilia of all ages.

When they focused on children and young adults (aged less than 25 years), the researchers found that the incidence rate of intracranial hemorrhage was much higher, at 7.4 per 1000 person-years, but the mortality rate was 0.5 per 1000 person-years. Finally, they found that the pooled cumulative intracranial hemorrhage incidence was 2.1% per 100 live births in neonates with hemophilia.

According to Zwagemaker and the co-authors of the study, precise estimates of intracranial hemorrhage incidence and mortality in patients with hemophilia may be important for patients, caregivers, and researchers as well as health policy-makers.

Intracranial hemorrhage is defined as bleeding between the brain and skull or within the brain tissue itself. It remains the most serious event in patients with hemophilia, resulting in high rates of disability and mortality. Early prophylaxis could reduce the risk of intracranial hemorrhagic events, as could close monitoring of early symptoms and identifying potential risk factors.


Zwagemaker AF, Gouw SC, Jansen JJ, et al. Incidence and mortality rates of intracranial hemorrhage in hemophilia: a systematic review and meta-analysis. Blood. Published online August 19, 2021. doi:10.1182/blood.2021011849