Risk Factors for Intracranial Hemorrhage in Hemophilia A Identified

Patients with hemophilia A who received prophylaxis had a reduced risk for intracranial hemorrhage (ICH), according to retrospective data from the ATHNdataset.

“ICH in persons with hemophilia A is associated with a high rate of mortality and morbidity,” study researchers explained. “ICH risk is 10–20 times higher in people with hemophilia (PWH) than the general population.”

Using the ATHNdataset, the researchers identified 7,837 people with hemophilia A aged two to 75 years.

During a median follow-up of 10.7 years, 1.7% of people in the study cohort had ICH. Incidence of ICH was significantly associated with age (P<.001), Medicaid insurance (P=.018), mortality status (P=.011), and having hepatitis C (P=.011), HIV (P<.001), or hypertension (P=.001). There were no associations between ICH and race or ethnicity.

The study found a significant increase in the incidence of ICH among patients who had never received prophylaxis (incidence rate ratio=0.63; 95% CI, 0.43-0.94; P=.020) and by inhibitor status (IRR=1.76; 95% CI, 0.97-3.20; P=.059).

Additionally, those people who had never received prophylactic treatment had significantly greater risk for developing ICH (hazard ratio=1.56; 95% CI, 1.06-2.30; P=.024).

“Results from multivariable analysis with an interaction term indicated being aged 13–74 years or never having had HIV were associated with lower risk of ICH, while ever being covered by Medicaid or never having received either (factor VIII) treatment or prophylactic treatment were associated with greater risk of ICH during the study period,” the researchers wrote.

Based on this, they concluded that “Medicaid-insured persons with hemophilia A could represent an important population for ICH risk mitigation.”

The researchers noted that their analysis did not corroborate previous reports that indicated inhibitor positivity was associated with increased risk of ICH; instead, they found inhibitor status was not associated with ICH in all analyses.

In all, the risk factors identified in this study—age from two to 12 years, Medicaid coverage, HIV, and hypertension, and never having received prophylactic or factor treatment—should be reevaluated as treatment of hemophilia evolves and use of nonfactor treatments and gene therapy increases.

Reference

Hu J, Chandler M, Manuel CM, et al. Risk of intracranial hemorrhage in persons with hemophilia A in the United States: real-world retrospective cohort study using the ATHNdataset. J Blood Med. 2024. doi:10.2147/JBM.S443380