Von Willebrand Factor, Deficiency Clinical Trial
Official title:
Low Von Willebrand in Ireland Cohort Study
The Low Von Willebrand in Ireland Cohort (LoVIC) study focuses on the bleeding phenotype and biological mechanisms underlying low Von Willebrand Factor (VWF) levels.
All patients with bleeding disorders in Ireland are registered on a national bleeding
disorder database and attend the National Coagulation Centre in St. James's Hospital, Dublin,
Ireland or the paediatric centre, Our Lady's Children's Hospital Crumlin for annual review.
At review eligible patients will be invited to participate in the Low Von Willebrand in
Ireland Cohort (LOVIC) study.
Following consent, an extensive bleeding assessment tool will be administered by a
coagulation haematologist to all participants from which the International Society of
Thrombosis and Haemostasis Bleeding Assessment Tool (ISTH BAT) and the Condensed Molecular
and Clinical Markers for the Diagnosis and Management of Type 1 Von Willebrands Disease
(MCMDM-1 VWD) scores can be derived. In addition, blood will be drawn for von Willebrand
factor (VWF) measurements, VWF propeptide, platelet VWF. Citrated plasma and DNA will be
stored for each patient. The relationship between laboratory parameters, (including von
Willebrand factor, platelet VWF, FVIII and concomitant coagulation disorders) and the
clinical phenotype in patients with low VWF will be studied. We will assess the effect of the
laboratory parameters on the severity of bleeding tendency. In the future mutation analysis
of the VWF gene will be performed in all participants in the LOVIC study.
Historical patient records and laboratory results will be reviewed and DDAVP
(1-desamino-8-D-arginine vasopressin) fall off studies documented where available. If no
previous DDAVP fall off study has been performed patients will be invited to attend.
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