A new study out of the UK found that only one-third of adults and two-thirds of children with severe hemophilia A and B (HA/HB) were free of hemarthroses in 2018, despite all patients reporting high treatment compliance.

The study, published in BMJ Open, utilized data from a patient-reported online diary, Haemtrack, and the Hemophilia Joint Health Score (HJHS) from the National Haemophilia Database to report the 12-month prevalence of joint bleeds among children and adults with severe hemophilia A and B without current inhibitors.

Read more about hemophilia types A, B, and C

“The fact that patients who were highly compliant with their prophylaxis regimen were included in our cohort and still only 70% of children and 30% of adults were bleed-free is concerning,” the research team wrote. “Understanding why these patients still experienced bleeding will be important to improving our ability to provide effective treatment and improve their overall functioning.”


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In children with HA, bleeding occurred most often in the knee, and in children with HB, it occurred most often in the ankle. In adults, most bleeding occurred in the ankle and elbow joints of those with HA and in the elbows of those with HB.

The median HJHS score for all children in the cohort was 0, suggesting little to no joint disease in the pediatric group or perhaps an inability of the HJHS to detect very early joint alterations after bleeding. In adults, total HJHS scores were higher overall for the ankle joint than for the knees or elbows, suggesting the ankle is the most affected joint in this patient group.

The researchers note the importance of further research into the impact of hemarthroses on patient functioning as well as a better understanding of the bleeding profiles of those who are less treatment-compliant. These investigations may reveal additional factors that affect joint biomechanics and quality of life of individuals with hemophilia, resulting in more targeted and personalized interventions for this patient group.

Reference

Wilkins RA, Stephensen D, Siddle H, et al. Twelve-month prevalence of haemarthrosis and joint disease using the Haemophilia Joint Health score: evaluation of the UK National Haemophilia Database and Haemtrack patient reported data: an observational study. BMJ Open 2022;12:e052358. doi:10.1136/bmjopen-2021-052358