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

25. september 2026 oppdatert av: Centre Hospitalier Universitaire de la Guadeloupe

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

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.

Studieoversikt

Status

Har ikke rekruttert ennå

Forhold

Detaljert beskrivelse

Sickle cell anemia is the most common genetic disease worldwide. It is due to a homozygous mutation in the beta globin gene leading to the production of abnormal hemoglobin S (HbS). This hemoglobin polymerizes and precipitates in the red blood cells (RBCs) under hypoxic conditions, leading to the rigidification of the red blood cells that obstruct the small vessels and are subsequently destroyed. The disease is thus characterized by chronic hemolytic anemia associated with painful vaso-occlusive crises (VOC) and chronic inflammation. The HbSC genotype is associated with anemia and less severe clinical manifestations than the HbSS genotype. However, the risk of venous thromboembolic events, particularly pulmonary embolism, is paradoxically higher. The pathophysiological mechanisms involved in the occurrence of VOC and venous thromboembolic events (VTE) have not been fully elucidated. They involve phenomena of ischemia-reperfusion and complex interactions between the RBC, the endothelium, the leucocytes and the platelets. The sickle RBCs express phosphatidylserine (PS) on their surface, which facilitates their adhesion to other blood cells and their interaction with the endothelium. The release of hemoglobin and free heme into the circulation, along with oxidative stress, induces endothelial activation and dysfunction, as well as vascular inflammation, platelet activation and clotting. Numerous studies have documented the existence of coagulation activation in sickle cell patients during the stable phase of the disease and during VOCs, decreased levels of physiological inhibitors of coagulation and resistance to activated protein C. One of the main mechanisms of coagulation activation in sickle cell patients is increased tissue factor expression on the surface of activated monocytes and endothelial cells. Free heme promotes NETs (neutrophil extracellular traps) formation, which activates the intrinsic coagulation pathway. Moreover, platelets probably play a pivotal role in the thrombo-inflammatory process, coagulation activation, and the occurrence of VOC and VTE. We hypothesize that sickle cell patients may exhibit a different thrombin generation profile as well as distinct platelet activation and reactivity profiles, depending on their SS or SC genotype.

Studietype

Observasjonsmessig

Registrering (Antatt)

60

Kontakter og plasseringer

Denne delen inneholder kontaktinformasjon for de som utfører studien, og informasjon om hvor denne studien blir utført.

Studiekontakt

Studiesteder

Deltakelseskriterier

Forskere ser etter personer som passer til en bestemt beskrivelse, kalt kvalifikasjonskriterier. Noen eksempler på disse kriteriene er en persons generelle helsetilstand eller tidligere behandlinger.

Kvalifikasjonskriterier

Alder som er kvalifisert for studier

18 år og eldre (Voksen, Eldre voksen)

Tar imot friske frivillige

Nei

Kjønn som er kvalifisert for studier

Alle

Prøvetakingsmetode

Ikke-sannsynlighetsprøve

Studiepopulasjon

Patients with sickle cell disease and the HbSS or HbSC genotype recruited from the participating study centers in Guadeloupe or Bobigny

Beskrivelse

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

Studieplan

Denne delen gir detaljer om studieplanen, inkludert hvordan studien er utformet og hva studien måler.

Hvordan er studiet utformet?

Designdetaljer

  • Observasjonsmodeller: COHORT
  • Tidsperspektiver: CROSS_SECTIONAL

Kohorter og intervensjoner

Gruppe / Kohort
SS Group

Patients with sickle cell disease and an SS genotype.
SC Group

Patients with sickle cell disease and an SC genotype.

Hva måler studien?

Primære resultatmål

Resultatmål
Tiltaksbeskrivelse
Tidsramme
Endogenous thrombin potential (ETP)
Tidsramme: At baseline

Thrombin generation profiles will be assessed in whole blood, plasma, and platelet-rich plasma. Endogenous thrombin potential will be compared between patients with sickle cell disease and an SS genotype and patients with sickle cell disease and an SC genotype
At baseline

Sekundære resultatmål

Resultatmål
Tiltaksbeskrivelse
Tidsramme
Lag time of thrombin generation
Tidsramme: At baseline

Thrombin generation will be assessed in whole blood, plasma, and platelet-rich plasma. Lag time will be compared between patients with sickle cell disease and the HbSS genotype and those with the HbSC genotype.
At baseline
Time to peak thrombin generation
Tidsramme: At baseline

Thrombin generation will be assessed in whole blood, plasma, and platelet-rich plasma. Time to peak will be compared between patients with sickle cell disease and the HbSS genotype and those with the HbSC genotype.
At baseline
Peak height of thrombin generation
Tidsramme: At baseline

Thrombin generation will be assessed in whole blood, plasma, and platelet-rich plasma. Peak height will be compared between patients with sickle cell disease and the HbSS genotype and those with the HbSC genotype.
At baseline
Platelet activation profile
Tidsramme: At baseline

Platelet activation profiles will be assessed and compared between patients with sickle cell disease and the HbSS genotype and those with the HbSC genotype.
At baseline
Platelet aggregation profile
Tidsramme: At baseline

Platelet aggregation profiles will be assessed and compared between patients with sickle cell disease and the HbSS genotype and those with the HbSC genotype.
At baseline

Samarbeidspartnere og etterforskere

Det er her du vil finne personer og organisasjoner som er involvert i denne studien.

Sponsor

Centre Hospitalier Universitaire de la Guadeloupe

Samarbeidspartnere

Hospital Avicenne

Etterforskere

  • Studieleder: Véronique Baccini, MD PhD, chu de la guadeloupe
  • Hovedetterforsker: Sylvain Lejeune, MD PhD, Centre de Référence Maladies Rares Adulte de Bobigny " Drépanocytose / syndromes drépanocytaires majeurs " de l'hôpital Avicenne
  • Hovedetterforsker: Maryse ETIENNE JULAN, MD, CHU de la Guadeloupe Coordonnateur, Centre de référence des syndromes drépanocytaires majeurs, thalassémies et autres pathologies rares du globule rouge et de l'érythropoïèse

Studierekorddatoer

Disse datoene sporer fremdriften for innsending av studieposter og sammendragsresultater til ClinicalTrials.gov. Studieposter og rapporterte resultater gjennomgås av National Library of Medicine (NLM) for å sikre at de oppfyller spesifikke kvalitetskontrollstandarder før de legges ut på det offentlige nettstedet.

Studer hoveddatoer

Studiestart (Antatt)

15. oktober 2026

Primær fullføring (Antatt)

15. oktober 2028

Studiet fullført (Antatt)

15. oktober 2028

Datoer for studieregistrering

Først innsendt

25. september 2026

Først innsendt som oppfylte QC-kriteriene

25. september 2026

Først lagt ut (Faktiske)

1. oktober 2026

Oppdateringer av studieposter

Sist oppdatering lagt ut (Faktiske)

1. oktober 2026

Siste oppdatering sendt inn som oppfylte QC-kriteriene

25. september 2026

Sist bekreftet

1. september 2026

Mer informasjon

Begreper knyttet til denne studien

Nøkkelord

Ytterligere relevante MeSH-vilkår

Andre studie-ID-numre

  • CHUG_RIPH2_2026/01

Plan for individuelle deltakerdata (IPD)

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NEI

Legemiddel- og utstyrsinformasjon, studiedokumenter

Studerer et amerikansk FDA-regulert medikamentprodukt

Nei

Studerer et amerikansk FDA-regulert enhetsprodukt

Nei

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