Contributions of von Willebrand factor to clinical severity of sickle cell disease: a systematic review and metanalysis

Aim To evaluate the contributions of VWF to the clinical manifestation and severity of SCDDesign A systematic review of peer-reviewed articles published in English. The review was carried out in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist.Methods...

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Main Authors: T. U. Nwagha, Martins Nweke, E. D. Ezigbo
Format: Article
Language:English
Published: Taylor & Francis Group 2022-12-01
Series:Hematology
Subjects:
Online Access:https://www.tandfonline.com/doi/10.1080/16078454.2022.2107908
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author T. U. Nwagha
Martins Nweke
E. D. Ezigbo
author_facet T. U. Nwagha
Martins Nweke
E. D. Ezigbo
author_sort T. U. Nwagha
collection DOAJ
description Aim To evaluate the contributions of VWF to the clinical manifestation and severity of SCDDesign A systematic review of peer-reviewed articles published in English. The review was carried out in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist.Methods The data sources for the review included MEDLINE, PubMed, CINAHL, and Academic Search Complete. Articles that applied a quantitative approach to the investigation of the relationship of vWF with clinical manifestations and severity indices were included. The risk of bias assessment was carried out with a mixed-method appraisal tool. We computed I 2 to estimate the degree of heterogeneity.Result There was a significantly higher level of VWF in SCD than in the control (d = 2.7, Z = 4.865, P < 0.001, I 2 = 96.41%). Significant positive correlations were obtained for the relationship of VWF with vasoocclusive crisis (r= 0.277, Z= 5.077, P < 0.001, 1 2 =15.62), rate of hemolysis (r=0.441; Z= 4.440, I 2 = <1%), extracellular haemoglobin (r=-0.397, Z=-4.155, I 2 =<1%) and CRP (r = 0.331, Z = 4.566, P < 0.001, I 2 < 1%).The VWF is important in determining the clinical severity of sickle cell disease, which constitutes a putative therapeutic target. More work is required to understand the causal direction underlying the association between VWF levels and the clinical severity of sickle cell disease and the potential role that VWF plays in the clinical manifestations of sickle cell disease.Protocol registration The protocol was registered with PROSPERO (CRD42021262625).
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spelling doaj.art-ee934844a0c84a41af991d07d68b8d822022-12-22T01:32:33ZengTaylor & Francis GroupHematology1607-84542022-12-0127186086610.1080/16078454.2022.2107908Contributions of von Willebrand factor to clinical severity of sickle cell disease: a systematic review and metanalysisT. U. Nwagha0Martins Nweke1E. D. Ezigbo2Department of Haematology, Faculty of Medicine, University of Nigeria /University of Nigeria Teaching Hospital Ituku Ozalla, Nsukka, NigeriaFledgelight Evidence Consult, Trans-Ekulu, NigeriaDepartment of Medical Laboratory Science, Faculty of Health Sciences, University of Nigeria Enugu Campus, Nsukka, NigeriaAim To evaluate the contributions of VWF to the clinical manifestation and severity of SCDDesign A systematic review of peer-reviewed articles published in English. The review was carried out in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist.Methods The data sources for the review included MEDLINE, PubMed, CINAHL, and Academic Search Complete. Articles that applied a quantitative approach to the investigation of the relationship of vWF with clinical manifestations and severity indices were included. The risk of bias assessment was carried out with a mixed-method appraisal tool. We computed I 2 to estimate the degree of heterogeneity.Result There was a significantly higher level of VWF in SCD than in the control (d = 2.7, Z = 4.865, P < 0.001, I 2 = 96.41%). Significant positive correlations were obtained for the relationship of VWF with vasoocclusive crisis (r= 0.277, Z= 5.077, P < 0.001, 1 2 =15.62), rate of hemolysis (r=0.441; Z= 4.440, I 2 = <1%), extracellular haemoglobin (r=-0.397, Z=-4.155, I 2 =<1%) and CRP (r = 0.331, Z = 4.566, P < 0.001, I 2 < 1%).The VWF is important in determining the clinical severity of sickle cell disease, which constitutes a putative therapeutic target. More work is required to understand the causal direction underlying the association between VWF levels and the clinical severity of sickle cell disease and the potential role that VWF plays in the clinical manifestations of sickle cell disease.Protocol registration The protocol was registered with PROSPERO (CRD42021262625).https://www.tandfonline.com/doi/10.1080/16078454.2022.2107908Von Willebrand factorsickle cell diseaseseverityclinical manifestationphenotype
spellingShingle T. U. Nwagha
Martins Nweke
E. D. Ezigbo
Contributions of von Willebrand factor to clinical severity of sickle cell disease: a systematic review and metanalysis
Hematology
Von Willebrand factor
sickle cell disease
severity
clinical manifestation
phenotype
title Contributions of von Willebrand factor to clinical severity of sickle cell disease: a systematic review and metanalysis
title_full Contributions of von Willebrand factor to clinical severity of sickle cell disease: a systematic review and metanalysis
title_fullStr Contributions of von Willebrand factor to clinical severity of sickle cell disease: a systematic review and metanalysis
title_full_unstemmed Contributions of von Willebrand factor to clinical severity of sickle cell disease: a systematic review and metanalysis
title_short Contributions of von Willebrand factor to clinical severity of sickle cell disease: a systematic review and metanalysis
title_sort contributions of von willebrand factor to clinical severity of sickle cell disease a systematic review and metanalysis
topic Von Willebrand factor
sickle cell disease
severity
clinical manifestation
phenotype
url https://www.tandfonline.com/doi/10.1080/16078454.2022.2107908
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