Evaluation of von Willebrand factor in Sickle Cell Disease at Steady State and in Vaso-occlusive Crisis
Huda Abedalameir Hussain, Zainab Wahab Al Maaroof, Abbas Fadhil HassonAbstract
Background:
Sickle cell disease (SCD) is the most common inherited hemoglobinopathy, involved the presence of hemoglobin S in red cells resulting from point mutations, causing replacement of glutamic acid by valine at position 6 of the beta-globin chain. The pathophysiology of sickle cell disease includes chronic inflammation, triggered by hemolysis and vaso-occlusion, which leads to variety of complications and elevated mortality. Hemostasis changes and thrombotic events are reported in response to leukocyte and endothelial activation.
Objective:
We aimed to assess thrombotic risk by measuring von Willebrand factor (vWF), ADAMTS13, and ADAMTS13 antibody during steady state and vaso-occlusive crisis.
Materials and Methods:
This case–control study included 80 individuals consisting of 40 patients with SCD and 40 matched controls enrolled from Babylon Teaching Hospital for maternity and children. Clinical information was obtained by drawing 5 mL of blood to measure the serum level of vWF, ADAMTS13, and anti-ADAMTS13 antibody by using the enzyme-linked immunosorbent assay kit.
Results:
There was a significant difference in vWF between patients and controls (
Conclusion:
The levels of vWF and anti-ADAMTS13 antibody were significantly higher in patients than in controls, but no significant changes were observed between patient subgroups. The level of ADAMTS13 Ag was lower in patients compared to controls, with no difference between patient subgroups.