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Background: Sickle cell disease occurs due to a mutation in β chains and the substitution of valine instead of glutamate in the sixth position of the ß-chain that causes polymerization and vascular blockage. The aim of this study was to compare the serum C, S proteins and factor V Leiden between sickle cell patients and the control group. Materials and Methods: In this case-control study, performed in Khuzestan province, Iran, from 2008 to 2009, C and S proteins as well as factor V Leiden activity were measured in 100 patients with sickle cell disease and compared with 50 patient in the control group that matched in age, gender and race. The type of sickle cell disease, hydroxyurea intake, blood transfusions, the level of HbF, age and gender were also analyzed. Results: Out of 100 sickle cell patients entering the study 47 patients were males and rests of them were females. The mean age of patients was 20.2± 1.03 years (Range 3 to 58 years). The level of protein C and S were low in 35% and 24% of patients respectively, but in controls both were normal (P<0.0001). Factor V Leiden was increased in 27% of patient and 4% of controls (P=0.001). A significant relationship was detected between protein S levels and patients’ age (P=0.02). Conclusion: Among patients with sickle cell disease, protein C and S levels are reduced and factor V Leiden activity is increased compared to controls, which might cause hyper-coagulation state among these patients. Keywords: Sickle cell disease, protein C, protein S, Factor V Leiden

Keywords: Sickle cell disease, protein C, protein S, Factor V Leiden

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