Sickle cell Sickle cell anemia
Conditions
Interventions
None listed
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: 1. High performance liquid chromatography confirmed diagnosis of HbSS or HbSb0-thalassemia genotype. 2. Written informed consent by the patient. 3. For healthy controls: HbAA genotype, race sex and age matched blood donors, written informed consent.
Exclusion criteria
Exclusion criteria: 1. Blood transfusion in the preceding three months. 2. Painful crisis/acute chest syndrome/stroke or other acute complications in the preceding 2 weeks. 3. Pregnancy, active cancer, active infection, connective tissue diseases. 4. For healthy controls: any current disease
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| 1. Comparison of the number of CEC in sickle cell patients to the number in healthy controls. | — |
Secondary
| Measure | Time frame |
|---|---|
| 2. To study the relationship of CEC to the history of clinical vaso-occlusion (e.g., the frequency of painful crises). 3. To analyze the relationship of CEC to established organ damage and dysfunction. 4. To determine the relationship of CEC to laboratory risk factors for poor outcome (e.g., the degree of anemia and the percentage of fetal haemoglobin). 5. To analyze the relationship of CEC to other biomarkers of disease activity (e.g. endothelial activation and fucntion, the hypercoagulable state and angiogenesis. | — |
Countries
Netherlands