No registrations found.
ID
Source
Brief title
Health condition
Sickle cell disease & β-thalassemia
Sponsors and support
Intervention
Outcome measures
Primary outcome
The primary outcome is the survival of transfused RBCs in patients with sickle cell disease and β-thalassemia.
Secondary outcome
Inter patient study parameters will focus on the effects of the transfusion. The main parameters will be:
• Inflammatory/immunomodulatory response on transfused RBCs.
• Influence of previous responder status on RBC survival.
• Antibody formation to the biotin label.
Intra patient study parameters will focus on the effect of matching of the RBC units. Both units will be matched differentially.
The main parameters will be:
• The recovery, short term and long term survival of both RBC units.
• The (immediate and late) host immune response to both RBC units by measuring effect on expression of markers of clearance and inflammation as well as transcriptome analysis.
Background summary
Rationale: Red blood cell (RBC) transfusions are currently one of the most important therapeutic options for patients with sickle cell disease and β-thalassemia. Over the last years much research has been conducted to improve transfusion safety, especially focussing on antigen matching to prevent alloimmunization. However, the fate of transfused RBC in these patients is largely unknown. Until now it has been difficult to track RBCs once transfused. A recently developed method (biotinylation of RBC units) allows a robust analysis of survival and clearance of the transfused RBCs. Insight in these phenomena may help to elucidate individual variation of RBC survival and behaviour in these patients.
Our objective is to assess the survival of transfused red blood cells (RBC) in patients with sickle cell disease and β-thalassemia by use of biotin labeled RBCs
Study objective
Survival of transfused erythrocytes will depent on donor and unit characteristics
Study design
T-1: Analysis of blood group genes
T0: Pre transfusion
T1: 10 minutes after transfusion
T2: 2 hours after transfusion
T3: 1 day after transfusion
T4: 10-12 days after transfusion
T5: 4-6 weeks after transfusion
Inclusion criteria
• Patient with either sickle cell disease (HbSS/HbSβ0/HbSC/HbSβ+) or β-thalassemia (major or transfusion dependent thalassemia intermedia)
• Currently on chronic transfusion program.
• >18 years of age
Exclusion criteria
No venous access for blood drawings
Design
Recruitment
IPD sharing statement
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 |
|---|---|
| NTR-new | NL7734 |
| Other | METC AMC : 2018_299 |