To determine the role of VWF and ADAMTS13 in the adherence of sickled erythrocytes to vascular endothelium.
ID
Source
Brief title
Condition
- Red blood cell disorders
- Congenital and hereditary disorders NEC
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
Main study parameter is the amount and percentage of adhesion as compared to a
baseline measurement with normal erythrocytes.
Secondary outcome
-
Background summary
Vascular occlusion causes devastating complications in patients with sickle
cell disease. However, the mechanisms of vascular occlusion are poorly
understood. It is hypothesized that disturbances in the ADAMTS13/VWF ratio
contribute to the pathophysiology of vascular occlusion in patients with sickle
cell disease.
This project aims to elucidate pathophysiology of vascular occlusion in
patients with sickle cell disease, focusing on disturbances in the balance
between VWF and ADAMTS13.
Study objective
To determine the role of VWF and ADAMTS13 in the adherence of sickled
erythrocytes to vascular endothelium.
Study design
In vitro study of the interactions between human umbilical vein endothelial
cells and sickled erythrocytes under continuous flow.
Study burden and risks
The first experiments will make use of the waste product of
erythrocytapheresis. This causes no increased risks associated with
participation. Since some blood cells may become overly activated after
apheresis, later experiments will make use of blood drawn from non-transfused
patients. This will entail taken 30 ml of blood extra, next to the normal
routine withdrawal. This volume is as small as possible to minimalise the
possible burden for the patient.
Meibergdreef 9
1105 AZ Amsterdam
Nederland
Meibergdreef 9
1105 AZ Amsterdam
Nederland
Listed location countries
Age
Inclusion criteria
Adult sickle cell patients with HbSS or HbS*0 als genotype
Exclusion criteria
Therapeutic intervention with hydroxyurea (Hydrea), with an extra exclusion criterium for the non-transfused patients that they have not been transfused in the last 4 months.
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL29705.018.09 |