Determination of von Willebrand factor level in patient with sickle cell diseasein vaso-occlusive crisis

Res Pract Thromb Haemost. 2020 Jul 10;4(5):902-905. doi: 10.1002/rth2.12378. eCollection 2020 Jul.

Abstract

Background: Sickle cell anemia (SCA) is a hypercoagulable state characterized by a significant alteration in hemostatic parameters that may predispose to increased risk of vaso-occlusive crisis (VOC). The role of von Willebrand factor (VWF) in the pathogenesis of VOC has not been fully investigated in Nigeria.

Objective: The objective of this study was to evaluate the level of VWF in subjects with sickle cell disease (SCD) in Calabar, Nigeria, to determine its role in the pathogenesis of VOC.

Methods: This was a comparative study carried out at the University of Calabar Teaching Hospital, Calabar, Nigeria. Sixty patients with SCA in VOC and 50 healthy controls were included. VWF levels were measured using Assaypro enzyme-linked immunosorbent assay kits.

Results: The mean age of patients with SCA in VOC and controls was 23.5 ± 7.2 years and 26.5 ± 5.6 years, respectively. The means (standard deviations) of VWF in patients in VOC and controls were 2.52 ± 0.34 IU/mL and 1.41 ± 0.23 IU/mL, respectively. There was no correlation of hematocrit and VWF in VOC (r = -0.034; P = .80), while there was a modest inverse correlation in controls.

Conclusions: Levels of VWFare elevated in a VOC state and thus may be implicated in the pathogenesis of VOC.

Keywords: sickle cell anemia; vaso‐occlusive crisis; von Willebrand factor.