Coagulation activation in sickle cell trait: an exploratory study.

Abstract

Recent epidemiologic data suggest that sickle cell trait (HbAS; AS) is a risk factor for venous thromboembolism. We conducted an exploratory study of healthy subjects with AS under baseline conditions to determine whether a chronic basal hyperactivation of coagulation exists, and if so, what mechanism(s) contribute to this state. Eighteen healthy AS individuals were compared to 22 African-American controls with a normal haemoglobin profile (HbAA; AA) and 17 patients with sickle cell disease (HbSS; SS). Plasma thrombin-antithrombin complexes and D-dimer levels were elevated in AS relative to AA patients (P = 0·0385 and P = 0·017, respectively), and as expected, were much higher in SSversusAA (P < 0·0001 for both). Thrombin generation in platelet poor plasma was indistinguishable between AA and AS subjects, whereas a paradoxical decrease in endogenous thrombin potential was observed in SS (P ≤ 0·0001). Whole blood tissue factor was elevated in SS compared to AA (P = 0·005), but did not differ between AA and AS. Plasma microparticle tissue factor activity was non-significantly elevated in AS (P = 0·051), but was clearly elevated in SS patients (P = 0·004) when compared to AA controls. Further studies in larger cohorts of subjects with sickle cell trait are needed to confirm the results of this preliminary investigation.

• Authors: Chirag Amin, Soheir Adam, Micah J Mooberry, Abdullah Kutlar, Ferdane Kutlar, Denise Esserman, Julia E Brittain, Kenneth I Ataga, Jen-Yea Chang, Alisa S Wolberg, Nigel S Key
• Journal: British journal of haematology (Br J Haematol) Vol. 171 Issue 4 Pg. 638-46 (Nov 2015) ISSN: 1365-2141 [Electronic] England
• PMID: 26511074 (Publication Type: Comparative Study, Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
• Copyright: © 2015 John Wiley & Sons Ltd.

Chemical References

• Cytokines
• Fibrin Fibrinogen Degradation Products
• Inflammation Mediators
• antithrombin III-protease complex
• fibrin fragment D
• Antithrombin III
• Fibrin
• Thromboplastin
• Peptide Hydrolases
• Thrombin

Topics

• Adult
• Black or African American
• Anemia, Sickle Cell (blood)
• Antithrombin III (analysis)
• Case-Control Studies
• Cell-Derived Microparticles (chemistry)
• Cytokines (blood)
• Female
• Fibrin (biosynthesis)
• Fibrin Fibrinogen Degradation Products (analysis)
• Humans
• Inflammation Mediators (blood)
• Male
• Middle Aged
• Peptide Hydrolases (analysis)
• Plasma
• Sickle Cell Trait (blood, complications)
• Thrombin (biosynthesis)
• Thrombophilia (blood, etiology)
• Thromboplastin (analysis)
• Venous Thromboembolism (etiology)