Blood outgrowth endothelial cells as a patient-derived ex vivo model system to study degranulation mechanisms in storage pool disease

Blood outgrowth endothelial cells as a patient-derived ex vivo model system to study degranulation mechanisms in storage pool disease - secREtion mechanisms in storage pooL disEASE (RELEASE study)

Status

Active, not recruiting

Phases

Unknown

Study type

Observational

Source

NL-OMON

Registry ID

NL-OMON42746

Enrollment

Registered

2016-02-24

Start date

2016-05-09

Completion date

Unknown

Last updated

2024-04-23

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

platelet function disorder storage pool disease

Interventions

None listed

Eligibility

Age

18 Years to 99 Years

Inclusion criteria

Inclusion criteria: - Age >= 18 years - Diagnosed with storage pool disease using standard luminoaggregometry

Exclusion criteria

Exclusion criteria: - Inability to give informed consent - Received a bone marrow or stem cell transplantation

Design outcomes

Primary

Measure	Time frame
Secretory responses of SPD platelets and BOECs as compared to healthy platelets and BOECs. Defects or abnormalities in Weibel-Palade bodies (WPB) biogenesis of SPD BOECs as compared to healthy BOECs. Alterations of SPD whole platelet and BOEC proteomes as compared to already established healthy platelet and BOEC proteomes	—

Secondary

Measure	Time frame
Mepacrine uptake and release of platelets of patients with storage pool disease as compared to healthy platelets Diagnostic utility of mepacrine uptake and release as compared to ATP/ADP ratio, electron microscopy, fluorescence microscopy and measurement of dense granule markers after activation using flow cytometry	—

Countries

Netherlands

Outcome results