Possible role of hemoglobin S in implicating hemostatic and inflammatory reactions: Study on Saudi Arabian population

Abstract

Background: Sickle cell disease (SS), is one of the most common inherited hematologic disorders, presents with chronic hemolytic anemia that can be punctuated by crises, infarcts, organ damage and hypercoagulable state. Sickle cell trait (AS) is a benign disorder. However, there is an increased risk for several abnormalities such as hematuria and pulmonary embolism. Coagulation and inflammation cannot be considered as two separate processes. Protein C is an important link between coagulation and inflammation; activated protein C can modulate inflammatory activity. Objectives: To examine the extent to which the coagulation activation and inflammatory link processes occur in hemoglobin SS disease (HbSS) patients in chronic state and in sickle cell trait (AS) individuals comparable with normal individuals. Subjects and Methods: Twenty sickle cell anemia patients in the chronic state and 20 AS individuals were included in this study. Coagulation tests and some acute phase reactants (inflammatory markers) were measured in all cases. Results: Comparison of SCD patients and AS individuals altogether, showed significantly higher prothrombin time, C-reactive protein and WVF activity (P < 0.05, P < 0.01 and P < 0.05, respectively) and significantly lower protein C level and protein S activity (P < 0.01 and P < 0.05, respectively) in HbSS patients than AS. Other parameters showed no significant difference. Conclusion: Some hemostatic and inflammatory link changes are present in SCD even in the chronic state and in AS individuals. Together with the known sickling of red blood cells, thrombocytosis, and increased viscosity, they contribute to the hypercoagulable state present in these individuals.

Keywords

• Antithrombin
• C-reactive protein
• fibrinogen
• protein C
• protein S
• sickle cell anemia