Dear Spiral user, We’re pleased to introduce Helix, https://helix.imperial.ac.uk/ Imperial’s new repository for research datasets. Please use Helix for all future dataset deposits. There is no change to Spiral - continue depositing as usual. For queries, contact the Research Data Management team at rdm-enquiries@imperial.ac.uk. Kind regards, the Spiral Team. The Spiral team
Elevated Factor VIII in hereditary haemorrhagic telangiectasia (HHT): association with venous thromboembolism
File(s)
Author(s)
Shovlin, C
Sulainam, N
Govani, FS
Jackson, JE
Begbie, ME
Type
Journal Article
Abstract
Introduction: Hereditary haemorrhagic telangiectasia (HHT) causes chronic nasal and gastrointestinal haemorrhage. Prothrombotic agents are commonly used for severe haemorrhage. Thrombotic risks have not been defined. Methods To identify prothrombotic variables in HHT patients, and assess their potential functional significance, a pilot ELISA-based study comparing plasma proteins in healthy individuals with HHT to age/sex-matched non-HHT controls was validated in a full study of 309 consecutive HHT-affected individuals. Results In the pilot study, Factor VIII (FVIII) and Von Willebrand Factor antigen concentrations were elevated in the HHT group compared to non-HHT controls (p0.0013, Mann-Whitney). Service laboratory measurements confirmed high FVIII:Ag in 125 HHT-affected individuals with no recent ill-health, intervention or venous thromboemboli. FVIII:Ag levels increased with age. Logistic regression also suggested an age-independent association with HHT-associated pulmonary arteriovenous malformations (PAVMs). No association was demonstrated between FVIII:Ag and acute phase response, disseminated intravascular coagulation, ABO group, pulmonary artery pressure, or markers of HHT haemorrhage. Elevated FVIII:Ag were associated with shortened activated partial thromboplastin times (APTTs), and VTE: VTE affected 20/309 (6.5%) HHT-affected individuals, at median age 61(36-71)yr. Four VTE occurred in Factor V Leiden heterozygotes in the months following PAVM-associated brain abscess. The strongest association with VTE was with log-transformed FVIII:Ag measured 10-132 months from VTE (odds ratio 2.41 (95% confidence intervals 1.254, 4.612, p=0.008). Age made no additional contribution to VTE risk once adjusted for FVIII:Ag. Conclusions HHT-related elevation of FVIII:Ag levels may influence thrombotic risk in HHT. Individualised risk-benefit considerations may be helpful in the management of individuals with HHT.
Date Issued
2007-10-11
Date Acceptance
2007-08-17
Citation
Thrombosis and Haemostasis, 2007, 98, pp.1031-1039
URI
DOI
ISSN
0340-6245
Publisher
Schattauer
Start Page
1031
End Page
1039
Journal / Book Title
Thrombosis and Haemostasis
Volume
98
Copyright Statement
© 2007 Schattauer GmbH, Stuttgart. This article is not an exact copy of the
original published article in Thrombosis and Haemostasis. The definitive publisher-authenticated version of Thromb Haemost. 2007 Nov;98(5):1031-9 is available online at: http://th.schattauer.de/en/contents/archive/issue/742/manuscript/8869.html
License URL
Publication Status
Published