Sickle cell anemia (SCA) is a hypercoagulable and prothrombotic disease state characterized by hemostatic perturbations that may predispose to increased risk of vaso-occlusive crisis (VOC) and other vasculopathic events. Von Willebrand Factor (vWF) has been shown to play an important role in the pathophysiology of vaso-occlusion in SCA, enhancing adhesive interactions with blood cells and the vascular endothelium. However, the levels of vWF in patients with SCA in Nigeria and its role in the pathophysiology of SCA vaso-occlusion have not been fully evaluated. The aim was to evaluate the plasma concentration of vWF in SCA patients in steady state and VOC for the purpose of determining its clinical value as marker of sickle cell VOC. This was an observational study carried out at the University of Uyo Teaching Hospital, Uyo, Nigeria. Eighty patients and 40 healthy HbAA volunteers were recruited for this study. Forty patients presented during steady state and the same number of patients presented during VOC. Hematocrit (HCT), white blood cell count (WBC), Platelet (PLT), prothrombin time (PT), activated partial thromboplastin time (APTT), and plasma concentration of vWF were determined. The mean values of vWF in VOC (3.85+/-3.81 IU/ml) and steady state (2.69 1.89 lU/ml) were higher than that of the control (1.53+/-0.40 IU/ml). There were statistically significant differences in HCT, WBC, PLT, PT, and APTT between the cases and HbAA control (p < 0.001). Levels of vWF are elevated in SCA during VOC and steady state and thus may serve as a potential marker to identify patients at risk of developing VOC.
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