Sickle Cell Anemia Sickle Cell Disease
Conditions
Interventions
None listed
Sponsors
Amsterdam UMC
Eligibility
Age
2 Years to 99 Years
Inclusion criteria
Inclusion criteria: Sickle Cell Disease >=6 years old Clinical need for an erythrocyte transfusion
Exclusion criteria
Exclusion criteria: - No informed consent - Pregnancy, self-reported - Active cancer - Chronic of acute HIV infection - Comorbid autoimmune/inflammatory disease - Pre-operative incidental transfusion
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| - Survival of donor RBCs in top up and exchange transfusions in SCD patients 4 weeks post-transfusion - Effects of patient-derived factors (genotype, HbS%, hemolysis, endothelial activation (proVWF/VWF ratio), inflammation, neutrophil activity/phenotype) on donor RBC survival | — |
Secondary
| Measure | Time frame |
|---|---|
| - Phenotypic changes in endogenous and donor RBCs after transfusion in SCD patients (1 hour, 24 hours, 1 week, 2 weeks, 3 and 4 weeks post-transfusion) as compared to pre-transfusion - Oxidative stress in endogenous/donor RBCs after transfusion in SCD patients as expressed by intracellular metabolomics (1 hour, 24 hours, 1 week, 2 weeks, 3 weeks and 4 weeks post-transfusion) - Immediate and late effects of transfusion on neutrophil activity and phenotype in SCD patients (1 hour, 24 hours, 1 week and 2 weeks post-transfusion) as compared to pre-transfusion values - Survival of endogenous RBCs 1, 2 and 4 weeks after transfusion measured by the ratio of glycine-15N and glycine 14N in heme | — |
Countries
Netherlands
Outcome results
None listed