In this study the cumulative incidence and the clinical and immunogenetic determinants of allo-antibody formation in SCD patients in response to RBC transfusion will be evaluated.
ID
Source
Brief title
Condition
- Haemoglobinopathies
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
The primary study parameters are:
- Immunogenetic determinants that could determine alloimmunisation; Variations
in the gene cluster encoding the Fc-gamma receptors and the SIRP-alpha receptor
(Signal Regulatory Protein-alpha).
- Clinical determinants that could determine alloimmunisation such as gender,
SCD genotype, geographic origin, age, age of first transfusion and number of
transfusions.
The main study outcome is the cumulative incidence of allo-antibody formation
in response to RBC transfusion.
Secondary outcome
N/A
Background summary
Allo-antibody formation is a frequent complication of transfusion. The risk of
alloimmunization varies from 1-8% in incidental transfusion recipients, to >40%
in patients receiving transfusion on a regular base. A population that
frequently receives transfusions is formed by patients with sickle cell disease
(SCD), a hereditary hemoglobinopathy characterized by chronic anemia. These
patients thus have an increased risk of developing allo-antibodies.
Red Blood Cell alloimmunization may jeopardize optimal transfusion support in
affected patients, poses these patients at risk for hemolytic transfusion
reactions, increases the costs of transfusion and forms a logistic challenge
for the blood supply system due to the need of extended blood type matching.
Both genetic and clinical factors are presumed to be risk factors for
alloimmunization. Genetic variation in the Fc-gamma-receptor was demonstrated
to influence antibody formation in patients with idiopathic thrombocytopenic
purpura.
Identifying risk factors will help in the identification of patients at high
risk of RBC alloimmunization. RBC alloimmunization may than be prevented by
special preventive measures such as a more extended matching strategies.
Study objective
In this study the cumulative incidence and the clinical and immunogenetic
determinants of allo-antibody formation in SCD patients in response to RBC
transfusion will be evaluated.
Study design
This is a national, multicenter observational cohort study that will include
SCD patients from three different hospitals.
Study burden and risks
The risks of participation are expected to be negligible. This is a
non-therapeutic study as the participating patients themselves will not benefit
from participating in this study. The burden is comparable with the burden that
patients with this disease experience in daily life with standard medical care
as the one-time only acquisition of a blood sample will be combined with
routine follow-up blood sampling.
Our research question is group related. As many SCD patients start receiving
transfusions in the first years of life, it is important to include children in
our study. Also, transfusion protocols have changed in the last years and
presently RBC units are matched more extensively than before. It is important
to include the effects of this strategy in our analyses. These effects can only
be addressed in children that have been transfused exclusively with extensively
matched RBC units.
Meibergdreef 9
Amsterdam 1105 AZ
NL
Meibergdreef 9
Amsterdam 1105 AZ
NL
Listed location countries
Age
Inclusion criteria
- Sickle Cell Disease (phenotypes HbSS, HbSC, HbS*+, HbS*0 or HbSE)
- History of at least 1 RBC transfusion in one of the participating centers
Exclusion criteria
None
Design
Recruitment
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 |
|---|---|
| CCMO | NL43029.018.13 |