Haemophilia
| Headline | |
|---|---|
| Etiology | deficiency of Factor VIII (A) or IX (B) X-linked recessive inheritance |
| Epidemiology | |
| Clinical presentation | haemarthrosis, mucocutaneous bleeding |
| Pathogenesis | |
| Diagnostic investigations | Clotting screen: APTT prolonged; PT normal FBC normal |
| Management | Prophylaxis - aim to prevent bleeding e.g. into joints - Emicizumab Bleeding into muscle compartments can lead to compartment syndrome |
the bleeding haemophiliac
- Replace the missing factor
- Antifibrinolytic agent = tranexamic acid