Constitutive hypercoagulability in pediatric sickle cell disease patients with hemoglobin SS genotype
© 2024 The Author(s)..
Background: Constitutive inflammation and hemostatic activation have been identified as key contributors to the pathophysiology of sickle cell disease (SCD), leading to clinical consequences such as vaso-occlusive crises and stroke. Patients with hemoglobin SS (HbSS) and hemoglobin SC (HbSC) genotypes are reported to have different symptoms, as do patients in steady-state and crisis situations. Differences among these groups remain unclear in pediatric patients.
Objectives: To compare hemostatic activity in HbSS and HbSC pediatric patients during steady state, in crisis, and in clinical follow-up and compare HbSS and HbSC patients with normal healthy children.
Methods: Whole-blood coagulation assay thromboelastography (TEG) was used to assess hemostatic activity. In parallel, flow cytometry was used to assess procoagulant surface expression of platelets and red blood cells.
Results: TEG results indicated no significant differences in clotting onset (R time), clot maximum amplitude, or maximum rate of thrombus generation among steady-state, crisis, and follow-up subgroups of HbSS and HbSC patients. TEG parameters did not differ significantly between HbSC patients and healthy children, while HbSS patients showed significantly shorter R time and greater maximum amplitude and maximum rate of thrombus generation, all indicative of a constitutive hypercoagulable state. Flow cytometry results did not detect increased platelet integrin αIIbβ3 activation or red blood cell procoagulant surface expression in SCD patients compared with unaffected children.
Conclusion: Our results indicate that pediatric SCD patients with the HbSS genotype have constitutively activated hemostasis relative to HbSC patients and healthy children. It remains to be determined how treatments that improve clinical outcomes in SCD patients affect this constitutively hypercoagulable state.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:8 |
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| Enthalten in: |
Research and practice in thrombosis and haemostasis - 8(2024), 3 vom: 13. März, Seite 102374 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Sussman, Raizl G [VerfasserIn] |
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| Links: |
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| Themen: |
Hypercoagulability |
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| Anmerkungen: |
Date Revised 25.04.2024 published: Electronic-eCollection Citation Status PubMed-not-MEDLINE |
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| doi: |
10.1016/j.rpth.2024.102374 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM370951387 |
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| 520 | |a © 2024 The Author(s). | ||
| 520 | |a Background: Constitutive inflammation and hemostatic activation have been identified as key contributors to the pathophysiology of sickle cell disease (SCD), leading to clinical consequences such as vaso-occlusive crises and stroke. Patients with hemoglobin SS (HbSS) and hemoglobin SC (HbSC) genotypes are reported to have different symptoms, as do patients in steady-state and crisis situations. Differences among these groups remain unclear in pediatric patients | ||
| 520 | |a Objectives: To compare hemostatic activity in HbSS and HbSC pediatric patients during steady state, in crisis, and in clinical follow-up and compare HbSS and HbSC patients with normal healthy children | ||
| 520 | |a Methods: Whole-blood coagulation assay thromboelastography (TEG) was used to assess hemostatic activity. In parallel, flow cytometry was used to assess procoagulant surface expression of platelets and red blood cells | ||
| 520 | |a Results: TEG results indicated no significant differences in clotting onset (R time), clot maximum amplitude, or maximum rate of thrombus generation among steady-state, crisis, and follow-up subgroups of HbSS and HbSC patients. TEG parameters did not differ significantly between HbSC patients and healthy children, while HbSS patients showed significantly shorter R time and greater maximum amplitude and maximum rate of thrombus generation, all indicative of a constitutive hypercoagulable state. Flow cytometry results did not detect increased platelet integrin αIIbβ3 activation or red blood cell procoagulant surface expression in SCD patients compared with unaffected children | ||
| 520 | |a Conclusion: Our results indicate that pediatric SCD patients with the HbSS genotype have constitutively activated hemostasis relative to HbSC patients and healthy children. It remains to be determined how treatments that improve clinical outcomes in SCD patients affect this constitutively hypercoagulable state | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a hypercoagulability | |
| 650 | 4 | |a sickle cell disease | |
| 650 | 4 | |a thromboelastography | |
| 700 | 1 | |a Mburu, Joy |e verfasserin |4 aut | |
| 700 | 1 | |a Steele, MacGregor |e verfasserin |4 aut | |
| 700 | 1 | |a Bang, Annie |e verfasserin |4 aut | |
| 700 | 1 | |a Friedman, Jeremy |e verfasserin |4 aut | |
| 700 | 1 | |a Goldman, Ran |e verfasserin |4 aut | |
| 700 | 1 | |a Kirby, Melanie |e verfasserin |4 aut | |
| 700 | 1 | |a Rand, Margaret L |e verfasserin |4 aut | |
| 700 | 1 | |a Blanchette, Victor S |e verfasserin |4 aut | |
| 700 | 1 | |a Pluthero, Fred G |e verfasserin |4 aut | |
| 700 | 1 | |a Williams, Suzan |e verfasserin |4 aut | |
| 700 | 1 | |a Kahr, Walter H A |e verfasserin |4 aut | |
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