Identifying fetuses at risk of intracranial hemorrhage (ICH) may aid in reducing its prevalence among infants and toddlers (ITs) with congenital hemophilia, according to research presented at the 2022 ASH Annual Meeting and Exposition.

Previous research has suggested that some clinical practices, such as avoiding head trauma during delivery, may reduce the risk of ICH; the early initiation of prophylaxis among those with a family history of bleeding disorders may also be beneficial. For this study, researchers evaluated whether mode of delivery, delivery practices, and initiation of prophylaxis, with a special focus on emicizumab, may be approached differently among individuals with a family history of bleeding disorders.

All data were obtained from the Community Counts Registry for Bleeding Disorders Surveillance. All included ITs were born in 2011 or later, were diagnosed with congenital hemophilia A or B, and had clotting factor activity levels of less than 50%.


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Data from 883 ITs were included in this analysis. Overall, 98% of patients were male, 73% were White, 78% had hemophilia A, 62% had severe disease, and 70% had a known family history of bleeding disorders.

Analysis showed that ITs with a family history of bleeding disorders were less likely to have had a vaginal delivery (67% vs 80% with no family history; P =.0006); these patients were also less likely to have an instrumental delivery (4% vs 9%, respectively; P =.02).

ITs with a family history of bleeding disorders were, however, more likely to be diagnosed earlier (P <.001), though there was no indication of different ages of prophylaxis initiation between these 2 groups.

Within the first 2 years of life, the ICH rate was 8%, with no significant difference between the present family history of bleeding disorder vs no family history groups. Further analysis showed that, among patients with an ICH, 12% were on continuous prophylaxis and 35% had an ICH within the first month of life. Of the patients with an ICH event within the first month of life, 71% had had a vaginal delivery and 33% had report of head trauma.

“The ICH prevalence in ITs is still substantial,” Jennifer H. Han, MD, of the Indiana Hemophilia and Thrombosis Center in Indianapolis, said when presenting the study’s findings. “There remains opportunity to prevent ICH by identifying at risk fetuses.”

Disclosure: Some study authors declared affiliations with biotech, pharmaceutical, or device companies. Please see the original reference for a full list of authors’ disclosures.

Reference

Han JH, Dupervil B, Kulkarni R, Mahajerin A, Thornburg CD. Clinical and treatment characteristics of infants and toddlers less than two years of age with hemophilia: a report from the Community Counts Registry. Presented at ASH 2022. December 10-13, 2022. Abstract 30.