Markers of coagulation activation in patients with hemoglobinopathy in Western Saudi Arabia

Abstract

Objective: This study aims to examine markers of coagulation activation and their possible clinical associations in sickle cell disease (SCD) and thalassemia. Materials And Methods: This study was conducted on patients with hemoglobinopathy followed up at King Abdulaziz University Hospital between October 2010 and November 2011. Demographic and clinical data were collected for all participants. The independent t-test was used to compare two group means, while the one-way analysis of variance test was used to compare more than two group means. Results: The study included 122 hemoglobinopathy cases and 34 controls. Protein C, protein S, antithrombin, and activated protein C resistance (APCR) were significantly lower in patients with hemoglobinopathy than in the control group, while D-dimer levels were significantly higher (P < 0.001 for all comparisons). Patients with SCD had significantly higher protein C and D-dimer levels than those who had thalassemia (P < 0.01 for both). Arterial and venous thromboses were more prevalent in patients with SCD (12.5 and 18.7%), compared to patients with thalassemia (9.3 and 2.3%, respectively). Cases had significantly lower protein C, protein S, antithrombin, and APCR values (P < 0.001 for each), and higher D-dimer levels (P = 0.016) than male controls. There was no significant difference in markers of coagulation activation between patients who had undergone splenectomy compared with those who had intact spleens. Conclusion: Natural anticoagulants were significantly lower, and D-dimer levels were higher in Saudi patients with SCD and thalassemia compared to healthy controls. The procoagulant phenotype was more pronounced in patients with SCD and was associated with a higher prevalence of clinical thrombosis, compared to patients with thalassemia in this population.

Keywords

• Coagulation activation
• sickle cell disease
• thalassemia