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The Many Facets of Acquired Haemophilia
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The Many Facets of Acquired Haemophilia

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My Presentation at the Royal Free Hospital Grand Round that was not seen because of AV troubles. Here's your chance.

My Presentation at the Royal Free Hospital Grand Round that was not seen because of AV troubles. Here's your chance.

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Transcript

  • 1. The many facets ofAcquired Haemophilia Wai Keong Wong Keith Gomez
  • 2. 79F• Recurrent Falls and Tiredness• R Distal Radius # + Swollen Left Knee• APTT 111 PT12 Fib 5.3 – APTT 50:50 Mix 37 (not fully corrected)• Hb 7.3
  • 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 ObjectivesImmune Find thesuppres Cause
  • 6. FEIBA rFVIIat½ 8-12 hrs t½ 2-3 hrs£4000 £3500
  • 7. Monitoring bypassing therapy Normal Baseline rFVIIa 90 ug/kg FEIBA 100 IU/kg 7
  • 8. Underlying Cause % Autoimmune disorder 14.1 Malignancy 11.5 Pregnancy 8.9 Drug-induced 3.3 Transfusion, dermatologic, infection, MGUS, other 7.8 Idiopathic 54.4Ref: Baudoet al.Blood July 5, 2012 vol. 120 no. 1 39-46
  • 9. 20 40 60 80 100 120 140 160 028/03/201104/04/201111/04/201118/04/201125/04/2011 Prednisolone 60mg02/05/201109/05/201116/05/2011 30mg23/05/201130/05/2011 Cyclophosphamide 50mg06/06/201113/06/201120/06/201127/06/201104/07/201111/07/201118/07/201125/07/201101/08/2011 ?Role for IVIg08/08/201115/08/2011 ?Role for Rituximab
  • 10. Complete remission dependent on treatment group. ○ Steroids alone □ Steroids and Cytotoxics Collins P W et al. Blood 2007;109:1870-1877©2007 by American Society of Hematology
  • 11. Inpatient Care ‘Routine’ Venepuncture PhysiotherapyObservations Line Insertion / Biopsies and Removal Surgery
  • 12. Venepuncture
  • 13. Manual Handling
  • 14. Sites of bleeding in patients with acquired hemophilia. Collins P W et al. Blood 2007;109:1870-1877 Unrelated to FVIII Level Contrast to Haemophilia A©2007 by American Society of Hematology
  • 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