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

1. The many facets of
Acquired 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
Objectives
Immune Find the
suppres Cause

6. FEIBA rFVIIa
t½ 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 54.4
Ref: Baudoet al.Blood July 5, 2012 vol. 120 no. 1 39-46

9. 20
40
60
80
100
120
140
160
0
28/03/2011
04/04/2011
11/04/2011
18/04/2011
25/04/2011
Prednisolone 60mg
02/05/2011
09/05/2011
16/05/2011
30mg
23/05/2011
30/05/2011
Cyclophosphamide 50mg
06/06/2011
13/06/2011
20/06/2011
27/06/2011
04/07/2011
11/07/2011
18/07/2011
25/07/2011
01/08/2011
?Role for IVIg
08/08/2011
15/08/2011
?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 Physiotherapy
Observations
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