3. On Examination
• R Arm Bruise from Axilla to Fingers
• Left Anterior CubitalFossa – Bruised
– Site of Venepuncture
• Bruising of the L Knee
• Hot Swollen R Knee
• BP 85/45 HR 120 RR 30 Sats 94%
4. Acquired Haemophilia
• Antibodies against Factor VIII
• Rare
– Prevalence 344 cases in UK
– Incidence 60-70 / yr 1-1.5 per million
– Median Age 74
• Diagnosis
– Prolonged APTT
– Does not correct with 50/50
– Low Factor VIII (IX rarely) + Inhibitor
5. Stop the
Bleed
Objectives
Immune Find the
suppres Cause
15. Were there warning signs?
• 2 weeks prior, mechanical fall and hit chin + R
distal radial #
– APTT 57
• Chin wouldn’t stop bleeding -> A+E
– Hb 11 APTT 87 -> GP to recheck
– Haem not consulted. Haem lab did not inform
Haem on-call
16. Conclusion
• Rare but important disorder
• Bleeding + High APTT
– 50/50 Mix does not correct
• Challenge of managing a rare condition
– Most bleeding episodes may be iatrogenic
– Cost £20000 / day on agents alone
• Management – Stop the Bleed/ Find the
underlying cause/ Immuno-suppress
Hinweis der Redaktion
Clotting Cascade ---- Simplified
Our lady had a eosinophilicpneumoniti, Autoimmune Screen, History and Clinical Examination, CT NCAP
There is a 20% chance of relapse?Less chance of Relapse with Rituximab?