Incidence of factor VIII inhibitors throughout life in severe hemophilia A in the United Kingdom

Hay, Charles R. M., Palmer, Ben, Chalmers, Elizabeth, Liesner, Ri, Maclean, Rhona, Rangarajan, Savita, Williams, Michael and Collins, Peter William ORCID: https://orcid.org/0000-0002-6410-1324 2011. Incidence of factor VIII inhibitors throughout life in severe hemophilia A in the United Kingdom. Blood 117 (23) , pp. 6367-6370. 10.1182/blood-2010-09-308668

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Abstract

The age-adjusted incidence of new factor VIII inhibitors was analyzed in all United Kingdom patients with severe hemophilia A between 1990 and 2009. Three hundred fifteen new inhibitors were reported to the National Hemophilia Database in 2528 patients with severe hemophilia who were followed up for a median (interquartile range) of 12 (4-19) years. One hundred sixty (51%) of these arose in patients ≥ 5 years of age after a median (interquartile range) of 6 (4-11) years' follow-up. The incidence of new inhibitors was 64.29 per 1000 treatment-years in patients < 5 years of age and 5.31 per 1000 treatment-years at age 10-49 years, rising significantly (P = .01) to 10.49 per 1000 treatment-years in patients more than 60 years of age. Factor VIII inhibitors arise in patients with hemophilia A throughout life with a bimodal risk, being greatest in early childhood and in old age. HIV was associated with significantly fewer new inhibitors. The inhibitor incidence rate ratio in HIV-seropositive patients was 0.32 times that observed in HIV-seronegative patients (P < .001). Further study is required to explore the natural history of later-onset factor VIII inhibitors and to investigate other potential risk factors for inhibitor development in previously treated patients.

Item Type:	Article
Date Type:	Publication
Status:	Published
Schools:	Medicine Systems Immunity Research Institute (SIURI)
Subjects:	R Medicine > R Medicine (General) R Medicine > RC Internal medicine
Publisher:	American Society of Hematology
ISSN:	0006-4971
Last Modified:	20 Oct 2022 08:27
URI:	https://orca.cardiff.ac.uk/id/eprint/28366

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