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Blood, 1 August 2007, Vol. 110, No. 3, pp. 815-825.
Prepublished online as a Blood First Edition Paper on April 19, 2007; DOI 10.1182/blood-2006-10-050435.


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Submitted October 17, 2006
Accepted April 2, 2007

Mortality rates, life expectancy and causes of death in people with haemophilia A or B in the United Kingdom who were not infected with HIV

Sarah C Darby*, Sau Wan Kan, Rosemary J Spooner, Paul LF Giangrande, Frank GH Hill, Charles RM Hay, Christine A Lee, Christopher A Ludlam, and Michael D Williams

Clinical Trial Service Unit, University of Oxford, Oxford, United Kingdom
Cancer Epidemiology Unit, University of Oxford, Oxford, United Kingdom
Haemophilia Centre, Churchill Hospital, Oxford, United Kingdom
Department of Haematology, Birmingham Children's Hospital NHS Trust, Birmingham, United Kingdom
Department of Haematology, Manchester Royal Infirmary, Manchester, United Kingdom
Haemophilia Centre, Royal Free Hospital, London, United Kingdom
Haemophilia Centre, Edinburgh Royal Infirmary, Edinburgh, United Kingdom

* Corresponding author; email: sarah.darby{at}ctsu.ox.ac.uk.

Since the 1970s mortality in the haemophilia population has been dominated by HIV and few reports have described mortality in uninfected individuals. This study presents mortality in 6018 people with haemophilia A or B in the UK during 1977-1998 who were not infected with HIV, with follow-up until 1/1/2000. Given disease severity and factor inhibitor status, all-cause mortality did not differ significantly between haemophilia A and haemophilia B. In severe haemophilia, all-cause mortality did not change significantly during 1977-1999. During this period it exceeded mortality in the general population by a factor of 2.69 (95% CI 2.37, 3.05) and median life-expectancy in severe haemophilia was 63 years. In moderate/mild haemophilia all-cause mortality did not change significantly during 1985-1999 and median life-expectancy was 75 years. Compared with mortality in the general population, mortality from bleeding and its consequences, and from liver diseases and Hodgkin's disease was increased, but for ischaemic heart disease it was lower, at only 62% (95% CI 51%, 76%) of general population rates, and for 14 other specific causes it did not differ significantly from general population rates. There was no evidence of any death from variant Creutzfeld-Jacob disease or from conditions that could be confused with it.


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