Glanzmann Thrombasthenia, Von Willebrand Diseases
Conditions
Keywords
constitutional haemorrhagic diseases, Videomicroscopy, prediction, bleeding, Videomicroscopy, prediction, bleeding
Brief summary
In Willebrand disease, there is currently no test available to identify non-invasively patients with a high risk of bleeding from angiodysplasias The study propose to use a sublingual capillary bed analysis by video-microscopy, a sensitive, reproducible and non-invasive technique, to assess whether sublingual capillary density is predictive of hemorrhagic risk for patients with von Willebrand disease.
Interventions
Measurement of sublingual capillary density by video microscopy
BIOLOGICALblood sample
Collection of citrate tubes of 5 mL at baseline and at 36 months
Sponsors
University Hospital, Lille
Study design
Allocation
NON_RANDOMIZED
Intervention model
PARALLEL
Primary purpose
DIAGNOSTIC
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Adult patient with a significant form of von Willebrand disease (according to the inclusion criteria of the Willebrand Disease French Reference Center), a Glanzmann Thrombasthenia, a moderate to severe Haemophilia A or a woman carrying the hemophilia gene * Social insured patient
Exclusion criteria
* Minor patient * Refusal of consent * Person benefiting from a system of legal protection * Pregnant patient
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| area under the ROC curve from Sublingual capillary density to inclusion for the event "existence of at least a clinically significant haemorrhage" | at 3 years |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Sublingual capillary density | At baseline | To compare the sublingual capillary density of patients with von Willebrand disease to that of 3 other groups of patients: * with a severe form of hemophilia A * with a moderate form of hemophilia A * women carrying the hemophilia gene |
| qualitative abnormalities of sublingual capillaries (capillary haemorrhage, capillary dystrophy) | At baseline and at 3 years | presence or absence of qualitative abnormalities |
| delta of Sublingual capillary density increase | between inclusion and end of study (at 3 years) | — |
| ISTH-BAT haemorrhagic score (International Society on Thrombosis and Hemostasis - Bleeding Assessment Tool) | At baseline, at 3 years | ISTH-BAT is a questionnaire minimum score ranges from 0 to 10 whereas maximum score ranges from 20 to 30 |
| delta of increase of ISTH-BAT haemorrhagic score | between inclusion and end of study (at 3 years) | ISTH-BAT is a questionnaire minimum score ranges from 0 to 10 whereas maximum score ranges from 20 to 30 |
| plasma levels of proangiogenic markers | at inclusion and end-of-study visits(at 3 years) | dosage of angiopoietin-1, angiopoetin-2 (pg/mL) measured by ELISA |
| plasma levels of galectine | at inclusion and end-of-study visits(at 3 years) | galectin-1 and galectin-3 measured by ELISA |
| plasma levels of VEGF | at inclusion and end-of-study visits(at 3 years) | Concentration (pg/ml) of Vascular Endothelial Growth Factor (VEGF) in blood measured by ELISA |
| delta of increase in plasma levels of all proangiogenic markers | At inclusion and end of study (at 3 years) | — |
Countries
France
Contacts
CONTACTAntoine Rauch, MD,PhD
PRINCIPAL_INVESTIGATORAntoine Rauch, MD,PhD
University Hospital, Lille
Outcome results
None listed