Evaluation Of Protein C Levels In Sickle Cell Disease Subjects Seen At The University Of Benin Teaching Hospital, Nigeria

Background: Protein C (PC) deficiency has been reported in sickle cell disease (SCD) and postulated to contribute to the pathogenesis as well as clinical manifestations of SCD. However there is paucity of data on PC in Nigerian patients with SCD. Objectives: This study was aimed at evaluating the levels of Protein C (PC) antigen and activity in SCD subjects; the prevalent type of PC deficiency and association between PC deficiency and parameters such as sex, age, ABO and Rh D blood groups. Patients/methods: A cross sectional study conducted at University of Benin Teaching Hospital, Nigeria. Twenty-nine consenting SCD subjects were consecutively recruited. Protein C antigen and activity levels were determined using immunological (ELISA) and functional assays (Protac method) respectively. Blood groups were determined using standard antisera. Results: Fifteen (51.7%) were males, 19 (65.5%) were <18 years, 18 (62.1%) had blood group O and 26 (89.7%) were Rh D positive. The mean PC antigen and activity levels were 65.7±4.9% and 48.8±2.8% of normal respectively. Type I PC deficiency was found in 6 (20.69%) and type II in 10 (34.48%) of the participants. Protein C antigen was significantly higher in subjects above 18 years (p=0.001). There was no significant association between PC deficiency and parameters such as sex, age, ABO and Rh D blood groups. Conclusions: Protein C levels were reduced in SCD subjects and functional PC deficiency was more prevalent. There was no significant association between PC deficiency and the assessed parameters (sex, age, ABO and Rh D blood groups).