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Blood, 1 March 2007, Vol. 109, No. 5, pp. 1870-1877.
Prepublished online as a Blood First Edition Paper on October 17, 2006; DOI 10.1182/blood-2006-06-029850.
 Previous Article | Next Article 
Submitted June 29, 2006
Accepted October 8, 2006
Acquired haemophilia A in the UK: a two year national surveillance study by UK Haemophilia Centre Doctors' Organisation
Peter W Collins*, Sybil Hirsch, Trevor P Baglin, Gerard Dolan, John Hanley, Michael Makris, David M Keeling, Ri Liesner, Simon A Brown, and Charles RM Hay
University Hospital of Wales and School of Medicine, Cardiff University, United Kingdom
Manchester Royal Infirmary, United Kingdom
Addenbrookes Hospital, United Kingdom
Queen's Medical Centre, United Kingdom
Royal Victoria Infirmary, United Kingdom
Hallamshire Hospital, United Kingdom
Oxford Haemophilia Centre, United Kingdom
Great Ormond Street Hospital, United Kingdom
Royal Free Hospital, United Kingdom
* Corresponding author; email: peter.collins{at}cardiffandvale.wales.nhs.uk.
Acquired haemophilia A is a severe bleeding disorder caused by an autoantibody to factor VIII. Previous reports have focused on referral centre patients and it is unclear whether these findings are generally applicable. To improve understanding of the disease, a two year observational study was established to identify and characterise the presenting features and outcome of all patients with acquired haemophilia A in the UK. This allowed a consecutive cohort of patients, unbiased by referral or reporting practice to be studied. A total of 172 patients with a median age of 78 years were identified, an incidence of 1.48/million/year. The cohort was significantly older than previously reported series, but bleeding manifestations and underlying diseases were similar. Bleeding was the cause of death in 9% of the cohort and remained a risk until the inhibitor had been eradicated. There was no difference in inhibitor eradication or mortality between patients treated with steroids alone and a combination of steroids and cytotoxics. Relapse of the inhibitor was observed in 20% of the patients who had attained first complete remission. The data provide the most complete description of acquired haemophilia A available and are applicable to patients presenting to all centres.
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