Comparison of Thrombin Generation, Platelet Activation and Aggregation Profiles in Patients With Sickle Cell Disease Depending on Their SS or SC Genotype (DrepAgreg)

Sickle cell disease (SCD) is characterized by chronic hemolytic anemia, painful crises called vaso-occlusive crises (VOC) and chronic inflammation. Activated platelets from SCD patients contribute to both chronic inflammation and painful VOC. Individuals with the HbSC genotype experience anemia and less severe clinical manifestations than those with the HbSS genotype; however, they are at higher risk of venous thromboembolic events, particularly pulmonary embolism. This study aims to investigate whether sickle cell patients exhibit different thrombin generation profiles as well as distinct platelet activation and aggregation profiles, depending on their SS or SC genotype.

Inclusion Criteria: * SCD patients with SS or SC * diagnosis of SCD performed by electrophoresis or high-performance liquid chromatography (HPLC) in a reference laboratory for hemoglobinopathies * patients older than 18 years at inclusion * clinically in a steady state at inclusion (no complication in the last month and no transfusion in the three last months) * patient followed up for SCD at the sickle cell center of Guadeloupe (University hospital of Guadeloupe, Pointe à Pitre) or at the Adult Rare Diseases Reference Center "Sickle Cell Disease / Major Sickle Cell Syndromes" of the Avicenne hospital in Bobigny. * patients who will provide written informed consent in accordance with the Declaration of Helsinki * patients affiliated with national social security Exclusion Criteria: * patients younger than 18 years old * patients with hemoglobinopathy other than SS and SC SCD * patients who have received transfusion therapy or on bleeding therapy for less than three months * patients no affiliated with national social security * pregnant or breastfeeding patients