When are children diagnosed as having severe haemophilia and when do they start to bleed? A 10-year single-centre PUP study

The aim of this single-centre study was to obtain data prospectively on whe n children are diagnosed as having severe haemophilia and when they start t o bleed. Results of this 10-year PUP study suggest that severe haemophilia is nowadays diagnosed much earlier than in the Sixties. Patients with sever e haemophilia (n = 37; FV III <0.01 U/ml) start to bleed at very different ages. While 44% of patients have their first bleeding episode within the fi rst year of life, others do not bleed before the age of four. The onset of joint bleedings generally occurs about half a year later than other types o f bleeding, While half our patients developed their first bleeding by the a ge of 1.22 years, the mean age for the first joint bleeding was 1.91 years. Conclusion Early-onset prophylactic therapy can prevent damage to the joint s, but for rational therapy the age at onset of bleeding must also be taken into account. A nonbleeding child does not benefit from prophylactic treat ment.