Association of coagulation activation with clinical complications in sickle cell disease

Kenneth I. Ataga, Julia Elizabeth Brittain, Payal Desai, Ryan May, Susan Jones, John Delaney, Dell Strayhorn, Alan Hinderliter, Nigel S. Key

Research output: Contribution to journalReview article

60 Citations (Scopus)

Abstract

Background: The contribution of hypercoagulability to the pathophysiology of sickle cell disease (SCD) remains poorly defined. We sought to evaluate the association of markers of coagulation and platelet activation with specific clinical complications and laboratory variables in patients with SCD. Design and Methods: Plasma markers of coagulation activation (D-dimer and TAT), platelet activation (soluble CD40 ligand), microparticle-associated tissue factor (MPTF) procoagulant activity and other laboratory variables were obtained in a cohort of patients with SCD. Tricuspid regurgitant jet velocity was determined by Doppler echocardiography and the presence/history of clinical complications was ascertained at the time of evaluation, combined with a detailed review of the medical records. Results: No significant differences in the levels of D-dimer, TAT, soluble CD40 ligand, and MPTF procoagulant activity were observed between patients in the SS/SD/Sβ0 thalassemia and SC/Sβ+ thalassemia groups. Both TAT and D-dimer were significantly correlated with measures of hemolysis (lactate dehydrogenase, indirect bilirubin and hemoglobin) and soluble vascular cell adhesion molecule-1. In patients in the SS/SD/Sβ0 thalassemia group, D-dimer was associated with a history of stroke (p = 0.049), TAT was associated with a history of retinopathy (p = 0.0176), and CD40 ligand was associated with the frequency of pain episodes (p = 0.039). In multivariate analyses, D-dimer was associated with reticulocyte count, lactate dehydrogenase, NT-proBNP and history of stroke; soluble CD40 ligand was associated with WBC count and platelet count; and MPTF procoagulant activity was associated with hemoglobin and history of acute chest syndrome. Conclusions: This study supports the association of coagulation activation with hemolysis in SCD. The association of D-dimer with a history of stroke suggests that coagulation activation may contribute to the pathophysiology of stroke in clinically severe forms of SCD. More research is needed to evaluate the contribution of coagulation and platelet activation to clinical complications in SCD.

Original languageEnglish (US)
Article numbere29786
JournalPLoS One
Volume7
Issue number1
DOIs
StatePublished - Jan 11 2012

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sickle cell anemia
Sickle Cell Anemia
Coagulation
coagulation
CD40 Ligand
Chemical activation
thalassemia
Platelets
stroke
platelet activation
Thalassemia
Platelet Activation
Thromboplastin
Stroke
hemolysis
Association reactions
pathophysiology
Hemolysis
lactate dehydrogenase
L-Lactate Dehydrogenase

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)
  • Agricultural and Biological Sciences(all)

Cite this

Ataga, K. I., Brittain, J. E., Desai, P., May, R., Jones, S., Delaney, J., ... Key, N. S. (2012). Association of coagulation activation with clinical complications in sickle cell disease. PLoS One, 7(1), [e29786]. https://doi.org/10.1371/journal.pone.0029786

Association of coagulation activation with clinical complications in sickle cell disease. / Ataga, Kenneth I.; Brittain, Julia Elizabeth; Desai, Payal; May, Ryan; Jones, Susan; Delaney, John; Strayhorn, Dell; Hinderliter, Alan; Key, Nigel S.

In: PLoS One, Vol. 7, No. 1, e29786, 11.01.2012.

Research output: Contribution to journalReview article

Ataga, KI, Brittain, JE, Desai, P, May, R, Jones, S, Delaney, J, Strayhorn, D, Hinderliter, A & Key, NS 2012, 'Association of coagulation activation with clinical complications in sickle cell disease', PLoS One, vol. 7, no. 1, e29786. https://doi.org/10.1371/journal.pone.0029786
Ataga KI, Brittain JE, Desai P, May R, Jones S, Delaney J et al. Association of coagulation activation with clinical complications in sickle cell disease. PLoS One. 2012 Jan 11;7(1). e29786. https://doi.org/10.1371/journal.pone.0029786
Ataga, Kenneth I. ; Brittain, Julia Elizabeth ; Desai, Payal ; May, Ryan ; Jones, Susan ; Delaney, John ; Strayhorn, Dell ; Hinderliter, Alan ; Key, Nigel S. / Association of coagulation activation with clinical complications in sickle cell disease. In: PLoS One. 2012 ; Vol. 7, No. 1.
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AU - Ataga, Kenneth I.

AU - Brittain, Julia Elizabeth

AU - Desai, Payal

AU - May, Ryan

AU - Jones, Susan

AU - Delaney, John

AU - Strayhorn, Dell

AU - Hinderliter, Alan

AU - Key, Nigel S.

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N2 - Background: The contribution of hypercoagulability to the pathophysiology of sickle cell disease (SCD) remains poorly defined. We sought to evaluate the association of markers of coagulation and platelet activation with specific clinical complications and laboratory variables in patients with SCD. Design and Methods: Plasma markers of coagulation activation (D-dimer and TAT), platelet activation (soluble CD40 ligand), microparticle-associated tissue factor (MPTF) procoagulant activity and other laboratory variables were obtained in a cohort of patients with SCD. Tricuspid regurgitant jet velocity was determined by Doppler echocardiography and the presence/history of clinical complications was ascertained at the time of evaluation, combined with a detailed review of the medical records. Results: No significant differences in the levels of D-dimer, TAT, soluble CD40 ligand, and MPTF procoagulant activity were observed between patients in the SS/SD/Sβ0 thalassemia and SC/Sβ+ thalassemia groups. Both TAT and D-dimer were significantly correlated with measures of hemolysis (lactate dehydrogenase, indirect bilirubin and hemoglobin) and soluble vascular cell adhesion molecule-1. In patients in the SS/SD/Sβ0 thalassemia group, D-dimer was associated with a history of stroke (p = 0.049), TAT was associated with a history of retinopathy (p = 0.0176), and CD40 ligand was associated with the frequency of pain episodes (p = 0.039). In multivariate analyses, D-dimer was associated with reticulocyte count, lactate dehydrogenase, NT-proBNP and history of stroke; soluble CD40 ligand was associated with WBC count and platelet count; and MPTF procoagulant activity was associated with hemoglobin and history of acute chest syndrome. Conclusions: This study supports the association of coagulation activation with hemolysis in SCD. The association of D-dimer with a history of stroke suggests that coagulation activation may contribute to the pathophysiology of stroke in clinically severe forms of SCD. More research is needed to evaluate the contribution of coagulation and platelet activation to clinical complications in SCD.

AB - Background: The contribution of hypercoagulability to the pathophysiology of sickle cell disease (SCD) remains poorly defined. We sought to evaluate the association of markers of coagulation and platelet activation with specific clinical complications and laboratory variables in patients with SCD. Design and Methods: Plasma markers of coagulation activation (D-dimer and TAT), platelet activation (soluble CD40 ligand), microparticle-associated tissue factor (MPTF) procoagulant activity and other laboratory variables were obtained in a cohort of patients with SCD. Tricuspid regurgitant jet velocity was determined by Doppler echocardiography and the presence/history of clinical complications was ascertained at the time of evaluation, combined with a detailed review of the medical records. Results: No significant differences in the levels of D-dimer, TAT, soluble CD40 ligand, and MPTF procoagulant activity were observed between patients in the SS/SD/Sβ0 thalassemia and SC/Sβ+ thalassemia groups. Both TAT and D-dimer were significantly correlated with measures of hemolysis (lactate dehydrogenase, indirect bilirubin and hemoglobin) and soluble vascular cell adhesion molecule-1. In patients in the SS/SD/Sβ0 thalassemia group, D-dimer was associated with a history of stroke (p = 0.049), TAT was associated with a history of retinopathy (p = 0.0176), and CD40 ligand was associated with the frequency of pain episodes (p = 0.039). In multivariate analyses, D-dimer was associated with reticulocyte count, lactate dehydrogenase, NT-proBNP and history of stroke; soluble CD40 ligand was associated with WBC count and platelet count; and MPTF procoagulant activity was associated with hemoglobin and history of acute chest syndrome. Conclusions: This study supports the association of coagulation activation with hemolysis in SCD. The association of D-dimer with a history of stroke suggests that coagulation activation may contribute to the pathophysiology of stroke in clinically severe forms of SCD. More research is needed to evaluate the contribution of coagulation and platelet activation to clinical complications in SCD.

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