HHE Haematology supplement 2018 | Page 15

Does intense prophylaxis always lead to the desired outcomes? Despite the plethora of data showing the advantages of standard rFVIII and rFIX therapies, prophylaxis with these products may delay versus prevent the onset of joint disease. A German study followed up 49 patients with haemophilia A for 28 years and revealed that they developed joint disease despite intensive prophylactic treatment, most prominently located in the ankles. Moreover, this linear pathological process only became apparent approximately ten years after treatment initiation. 25 A cross-sectional study using magnetic resonance imaging to evaluate joint status in patients with severe haemophilia A, a