RESUMO
Sickle cell disease [SCO] is due to beta 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 SCO, recently.The aim of this study was to determinate the differences of serum C and S protein and factor V leiden between sickle cell patients and control subjects. In this randomized case-control study, protein C, protein S and factor V leiden activity were measured in 100 SCO 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. 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<0.00l] in 27% of patient and 4% of controls. A significant [P=0.02] correlation was detected between protein S levels and age groups. Protein C and protein S levels reduced and factor V leiden activity increased in SCD patients and cause hypercoagulable state in these patients