Backgraund and Objective:Sickle cell disease (SCD) is due to β chain mutation and substitution of valine for glutamic acid in sixth position,that is cause increasing polymerization and vaso-occlusion. Decrease of protein C, protein S and increase in factor V leiden activity contribute to hypercoagulation state in SCD, recently.The aim of this study was to determinate the differences of serum C & S protein and factor V leiden between sickle cell patients and control subjects. Subjects and Methods: In this randomized case-control study, protein C, protein S and factor V leiden activity were measured in 100 SCD patients in crisis phase. And were compared between 50 age- gender - race- matched controls and SCD patients in, hydroxyurea intake, blood transfusion, levels of HbF, age and gender by Chi-Square and Anova statistical tests in SPSS software.Results: In 100 patients 47 were males and 53 females , mean age was20.2 (range 3-58, 1SD± 1.03). Protein C and protein S levels were significantly low (both P<0.0001) in 35% and 24% patient; respectively. But in controls it was not like this. Factor V leiden was increased significantly (P=0.001) in 27% of patient and 4% of controls. A significant (P=0.02) correlation was detected between protein S levels and age groups .Conclusion: Protein C and protein S levels reduced and factor V leiden activity increased in SCD patients and cause hypercoagulable state in these patients. Sci Med J 2011; 10(3):249-25
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