Sickle Cell Disease
Conditions
Keywords
Sickle cell disease, Allo-immunization
Brief summary
Sickle cell patients have a high prevalence of alloimmunization. This high rate of alloimmunization can be partially explained by the existence of an antigenic difference between the predominantly Caucasian donor population and the sickle cell patients of African origin. Genetic and environmental risk factors have also been described. The main risk factors that have been shown in retrospective or cross-sectional studies are some HLA alleles, the age of the patient, the number of leukocyte-depleted erythrocyte concentrates (CED) transfused, the number of transfusion episodes, the age of the CEDs, the existence of an inflammatory event at the time of transfusion and the presence of anti-erythrocyte autoantibodies.There is also evidence of an impaired TH response but the underlying immunological mechanism is not fully understood. The aim of this study is to study the prevalence and the risk factors for anti-erythrocyte alloimmunization in pediatric and adult patients with Sickle Cell Disease (with a SS genotype) who are being followed at Queen Fabiola University Children's Hospital (HUDERF) and at the CHU Brugmann Hospital. The identification of risk factors would allow the investigators to improve, or at least adapt, their transfusion policy to certain clinical or immuno-haematological situations.
Interventions
OTHERMedical file data collection
The information described in the 'outcome measures' section will be collected from the medical files of the patients.
Sponsors
Hanane EL KENZ
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Healthy volunteers
No
Inclusion criteria
\- Sickle cell disease patients (HbSS genotype) with a history of blood transfusions within the CHU Brugmann and the Queen Fabiola University Hospitals.
Exclusion criteria
\- None
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Date of birth | january 2013-december 2017 | Date of birth |
| Sex | january 2013-december 2017 | Sex |
| Blood group | january 2013-december 2017 | Blood group |
| Extended phenotype | january 2013-december 2017 | Sickle cell disease extended phenotype |
| Antibodies | january 2013-december 2017 | Presence/absence of irregular anti-erythrocytes antibodies (RAI) |
| Number of blood transfusions | january 2013-december 2017 | Number of blood transfusions |
| Auto antibodies | january 2013-december 2017 | Presence/absence of auto anti-erythrocytes antibodies (RAI) |
| Pathology | january 2013-december 2017 | Medical issue causing the patient to be included in a chronic blood transfusion program |
| Duration of the chronic transfusion program | january 2013-december 2017 | Duration of the chronic transfusion program |
Countries
Belgium
Outcome results
None listed