Study of Erythrocyte Parameters and Hypercoagulability in Sickle Cell Disease (SCD-TGA)

NCT05376046 | Recruiting DMC

• 1 other identifier: 2021/0328/OB
• Study Type: observational
• Target: 200
• Locations: 1 country
• Sites: 1

Brief Summary

Sickle cell disease (SCD) is an inherited haemoglobinopathy disorder caused by mutations in HBB gene with amino-acid substitution on β globin chain. The consequence is synthesis of altered haemoglobin S (HbS) which polymerises in red blood cell (RBC) at deoxygenated state. SCD is associated with chronic haemolytic anaemia, vaso-occlusive crisis (VOC) leading to frequent hospitalisation. The aim of the study was to to investigate whether a combination of routine laboratory biomarkers of haemolysis could be used to predict VOC development in confirmed SCD patients.

Conditions

Sickle Cell Disease; Vaso-occlusive Crisis

Keywords

sickle cell disease; vaso-occlusive crisis; reticulocyte count; thrombin generation assay; hypercoagulability

Outcome Measures

Primary Outcomes (1)

• Hospitalisation for Vaso-occlusive crisis within one years

  Following injury consultation, evaluation of biological markers predicting vaso-occlusive crisis requiring hospitalisation in the year

  1 year

Study Arms (2)

Sickle cell disease

Confirmed sickle cell disease withHaemoglobin profile was determined by high performance liquid chromatography (HPLC) (Variant II Biorad, California, United States), by capillary electrophoresis on Capillarys 3 Octa® (Kit hydragel hémoglobine Sebia, Lisses, France) and iso-electrofocalisation. Patients were included during injury evaluation in our tertiary centre.

Biological: Erythrocytic parameters and thrombin generation assay measurement

Healthy

25 healthy controls matched on age and gender

Biological: Erythrocytic parameters and thrombin generation assay measurement

Interventions

Erythrocytic parameters and thrombin generation assay measurement BIOLOGICAL

Erythrocytic parameters and thrombin generation assay measurement

Healthy; Sickle cell disease

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

All patients in the study were diagnosed and treated for SCD at Rouen University Hospital. Patients were included during annual visit in our tertiary centre. Patients with VOC were included less than 24hours after admission to emergency department. All patients treated with hydroxyurea have been treated for at least three years. All patients received a systematic annual visit to determine VOC development in the year. Patients were analyzed in four subgroups based on genotype and clinical status: * homozygous SCD (S/S) or β0 thalassemia (S/β0) at steady state; * homozygous SCD with α3.7 thalassemia (S/Sα3.7) at steady state; * heterozygous SCD with C haemoglobin (S/C) or β+ thalassemia (S/β+)at steady state; * patients hospitalized for VOC with any genotype.

You may qualify if:

• Sickle cell disease

You may not qualify if:

• \<18 years
• pregnancy
• Patient under protective guardianship or curatorship

Sponsors & Collaborators

• BILLOIR: lead

Study Sites (1)

Rouen university Hospital

Rouen, 76000, France

RECRUITING

Related Publications (1)

• Feugray G, Grall M, Gillardin B, Burdeau J, Ozanne N, Dumesnil C, Fauvel C, Billoir P. Hemoglobin and High-Density Lipoprotein as Biomarker of Left Atrial Dilatation in Sickle Cell Disease. EJHaem. 2025 Aug 22;6(4):e70135. doi: 10.1002/jha2.70135. eCollection 2025 Aug.

  PMID: 40862241 DERIVED

MeSH Terms

Conditions

Anemia, Sickle Cell; Vaso-Occlusive Crises; Thrombophilia

Condition Hierarchy (Ancestors)

Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Anemia; Hematologic Diseases; Hemic and Lymphatic Diseases; Hemoglobinopathies; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Target Duration: 5 Years
• Sponsor Type: OTHER
• Responsible Party: SPONSOR INVESTIGATOR
• PI Title: Associate Professor

Study Record Dates

• First Submitted: April 29, 2022
• First Posted: May 17, 2022
• Study Start: September 1, 2018
• Primary Completion: September 1, 2025
• Study Completion (Estimated): September 1, 2026
• Last Updated: May 24, 2022

Record last verified: 2022-05

Locations

FR (1)