Hemophilia

1. HAEMOPHILIA
A Coagulation Disorder

2. HAEMOPHILIA A
• Factor VIII deficiency
• X-linked recessive

3. PRESENTATION
• Depends on severity
• Early on life
• After surgery/trauma
• Bleeding into joints – crippling arthropathy
• Bleeding into muscle – haematoma
• Increase pressure can lead to nerve palsies and compartment syndrome

4. TESTS
• APTT prolonged
• Low factor VIII assay

5. MANAGEMENT
• Seek expert advice
• Avoid NSAIDS and IM injections
• Minor bleeding
• Pressure and elevation of the part
• Desmopressin (0.3ug/kg/12h IVI over 20 mins)
• Raised factor VIII levels
• Major bleeding (haemarthrosis)
• Raised factor VIII to 50% of normal
• Life threatening bleeds (obstructing airway)
• Raised factor VIII to 100% of normal

6. HEMOPHILIA B
• Known as Christmas disease
• Factor IX deficiency
• X-linked recessive
• Clinically same like hemophilia A

7. COAGULATION PATHWAY