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

Study of Erythrocyte Parameters and Hypercoagulability in Sickle Cell Disease

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.

Study Overview

• Status: Recruiting
• Conditions: Sickle Cell Disease; Vaso-occlusive Crisis
• Intervention / Treatment: Biological: Erythrocytic parameters and thrombin generation assay measurement

• Study Type: Observational
• Enrollment (Anticipated): 200

Contacts and Locations

Study Locations

• France
  • Rouen, France, 76000
    • Recruiting
    • Rouen University Hospital
    • Contact: Paul Billoir, PhD, associate professor; Phone Number: +33232888129; Email: paul.billoir@chu-rouen.fr

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• 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.

Description

Inclusion Criteria:

• Sickle cell disease

Exclusion Criteria:

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

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
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; Erythrocytic parameters and thrombin generation assay measurement
Healthy; 25 healthy controls matched on age and gender	Biological: Erythrocytic parameters and thrombin generation assay measurement; Erythrocytic parameters and thrombin generation assay measurement

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
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

Collaborators and Investigators

• Sponsor: BILLOIR

Study record dates

Study Major Dates

• Study Start (Actual): September 1, 2018
• Primary Completion (Anticipated): September 1, 2025
• Study Completion (Anticipated): September 1, 2026

Study Registration Dates

• First Submitted: April 29, 2022
• First Submitted That Met QC Criteria: May 10, 2022
• First Posted (Actual): May 17, 2022

Study Record Updates

• Last Update Posted (Actual): May 24, 2022
• Last Update Submitted That Met QC Criteria: May 17, 2022
• Last Verified: May 1, 2022

More Information

Terms related to this study

• Keywords: vaso-occlusive crisis; sickle cell disease; hypercoagulability; thrombin generation assay; reticulocyte count
• Additional Relevant MeSH Terms: Hematologic Diseases; Genetic Diseases, Inborn; Anemia; Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Hemoglobinopathies; Anemia, Sickle Cell; Thrombophilia; Hemostatics; Coagulants; Thrombin
• Other Study ID Numbers: 2021/0328/OB

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: Undecided

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No