Hemophilia Burden of Disease

In addition to causing visible bleeding and bruising, hemophilia can cause bleeding into joints and muscles, which may be subclinical.¹

Severity

The severity of bleeding manifestations in hemophilia generally correlates with the degree of the clotting factor deficiency.^1,2

Adapted from Srivastava A, et al. 2020.

Although residual FVIII/FIX activity level accounts for around 70% of the bleeding phenotype, the remaining 30% are potentially related to other unexplained individual variables.³

Sites of Bleeding

Hemophilia not only causes visible bleeding and bruises, which sometimes require surgical intervention, but also causes internal bleeding. Indeed, most bleeding in hemophilia occurs internally, into the muscles or joints.¹

Acute joint bleeds can cause permanent damage to the joints or hemophilic arthropathy.¹
Bleeding into the abdomen or brain can be serious or life-threatening.⁴

Common Locations of Muscle and Joint Bleeds⁵

Adapted from World Federation of Hemophilia.⁵

In addition to these complications, some patients with hemophilic arthropathy may experience acute to chronic pain.⁶ A survey of 119 Italian patients (including 76% with severe hemophilia) found that 61% of the patients reported experiencing pain; 71% qualified this pain as ‘chronic’, 69% as ‘acute’ and 8% as ‘postoperative’, and the pain was considered ‘severe’ in 65% of cases.⁶​​​​​​​

FVIII, factor VIII; FIX, factor IX.

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