Platelet behaviour in Hereditary Thrombocytopathies. An observational study on the platelet characteristics of a known bleeding disorder with two novel instruments.

Completed

Conditions: Congenital thrombocytopathy
hereditary platelet disorder
10035534

Registration Number: NL-OMON37561

Lead Sponsor: niversitair Medisch Centrum Utrecht

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Completed

Sex: Not specified

Target Recruitment: 56

Inclusion Criteria

1) Confirmed diagnosis of Storage Pool Disease or other hereditary thrombocytopathy.
2) 18 years of age or older

Exclusion Criteria

1) Uncertainty about diagnosis expressed by their treating physician.
2) Use of medication that is known to influence platelets such as acetylsalicylic acid,
clopidogrel, dipyridamole and NSAIDs (Non-Steroidal Anti-Inflammatory Drugs) within 14 days prior to inclusion.
3) Younger than the age of 18.

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>1) Platelet reactivity with increasing concentrations of agonist with or<br /><br>without an added constant concentration of antagonist. Activation is measured<br /><br>with Fluorescence Activated Cell Sorting (FACS) analysis and expressed in<br /><br>percentage positive, Median Fluorescence Intensity (MFI), the concentration of<br /><br>agonist which leads to half maximum effect (EC50) and Area Under the dose<br /><br>response Curve (AUC).<br /><br>2) Platelet aggregate formation measurements will be performed using a<br /><br>perfusion flow model. The main steps in platelet aggregate formation (adhesion,<br /><br>aggregation and spreading) will be studied and quantified. </p><br>

Secondary Outcome Measures

Name	Time	Method
<p>The secondary parameter/outcome is to study the correlation between the newly<br /><br>developed the ISTH/SSC Bleeding Assessment Tool and outcomes of FACS and<br /><br>Perfusion measurements. </p><br>