ISSN 2997-1036
International Journal of Hematology | Vol. 3, No. 2, February 2012 | pp. 9–16
DOI: 10.46882/2012/IJH/000026
Original Article
Title: Association between plasma von Willebrand factor antigen levels and vaso-occlusive crises in sickle cell nephropathy
Names of Authors: E. F. Chinedu¹, G. H. Haruna², I. J. Balogun³
Authors’ Affiliations: ¹Department of Haematology, University of Nigeria Teaching Hospital, Enugu, Nigeria; ²Department of Medicine and Nephrology, Lagos State University Teaching Hospital, Ikeja, Nigeria; ³Department of Chemical Pathology, University of Ilorin, Ilorin, Nigeria
Abstract: Endothelial activation and microvascular dysfunction drive the development of sickle cell nephropathy. This case-control study investigated the association between plasma von Willebrand factor antigen levels, microalbuminuria, and the frequency of acute painful crises in 80 patients with sickle cell anemia (HbSS) and 40 healthy hemoglobin AA controls. Plasma von Willebrand factor antigen concentrations were quantified using an enzyme-linked immunosorbent assay. Patients with sickle cell anemia exhibited significantly higher steady-state von Willebrand factor antigen levels (184.5 ± 32.4%) compared to controls (94.2 ± 12.6%, P < 0.001). Among the sickle cell cohort, patients with microalbuminuria (urine albumin-to-creatinine ratio 30 to 300 mg/g) showed a substantial elevation in von Willebrand factor antigen levels compared to those with normoalbuminuria (212.6% versus 161.4%, P < 0.01). Furthermore, von Willebrand factor antigen levels correlated positively with the annual frequency of vaso-occlusive crises (r = 0.54, P < 0.01). Elevated plasma von Willebrand factor antigen acts as a biomarker for endothelial stress, correlating with microvascular renal injury and severe vaso-occlusive phenotypes in sickle cell disease.
Keywords: Sickle cell nephropathy, von Willebrand factor, endothelial activation, microalbuminuria, vaso-occlusive crisis
Manuscript Timeline: Received: November 02, 2011; Revised: December 14, 2011; Accepted: January 05, 2012; Published: February 14, 2012