ADAMTS-13-VWF axis in sickle cell disease patients.

Autor: Ladeira VS; Campus Centro Oeste Dona Lindu, Universidade Federal de São João del-Rei, Rua Sebastião Gonçalves Coelho, 400 - Chanadour, Divinópolis, MG, CEP: 35501-296, Brazil.; Fundação Hemominas, Belo Horizonte, Minas Gerais, Brazil., Barbosa AR; Campus Centro Oeste Dona Lindu, Universidade Federal de São João del-Rei, Rua Sebastião Gonçalves Coelho, 400 - Chanadour, Divinópolis, MG, CEP: 35501-296, Brazil., Oliveira MM; Campus Centro Oeste Dona Lindu, Universidade Federal de São João del-Rei, Rua Sebastião Gonçalves Coelho, 400 - Chanadour, Divinópolis, MG, CEP: 35501-296, Brazil.; Fundação Hemominas, Belo Horizonte, Minas Gerais, Brazil., Ferreira LGR; Campus Centro Oeste Dona Lindu, Universidade Federal de São João del-Rei, Rua Sebastião Gonçalves Coelho, 400 - Chanadour, Divinópolis, MG, CEP: 35501-296, Brazil., de Oliveira Júnior WV; Campus Centro Oeste Dona Lindu, Universidade Federal de São João del-Rei, Rua Sebastião Gonçalves Coelho, 400 - Chanadour, Divinópolis, MG, CEP: 35501-296, Brazil., de Oliveira Renó C; Campus Centro Oeste Dona Lindu, Universidade Federal de São João del-Rei, Rua Sebastião Gonçalves Coelho, 400 - Chanadour, Divinópolis, MG, CEP: 35501-296, Brazil., Reis EA; Faculdade de Farmácia, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil., Chaves DG; Fundação Hemominas, Belo Horizonte, Minas Gerais, Brazil., Dusse LMS; Faculdade de Farmácia, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil., Dos Santos HL; Campus Centro Oeste Dona Lindu, Universidade Federal de São João del-Rei, Rua Sebastião Gonçalves Coelho, 400 - Chanadour, Divinópolis, MG, CEP: 35501-296, Brazil., de Barros Pinheiro M; Campus Centro Oeste Dona Lindu, Universidade Federal de São João del-Rei, Rua Sebastião Gonçalves Coelho, 400 - Chanadour, Divinópolis, MG, CEP: 35501-296, Brazil. melinapinheiro@ufsj.edu.br., Rios DRA; Campus Centro Oeste Dona Lindu, Universidade Federal de São João del-Rei, Rua Sebastião Gonçalves Coelho, 400 - Chanadour, Divinópolis, MG, CEP: 35501-296, Brazil. danyelleromana@ufsj.edu.br.
Jazyk: angličtina
Zdroj: Annals of hematology [Ann Hematol] 2021 Feb; Vol. 100 (2), pp. 375-382. Date of Electronic Publication: 2021 Jan 06.
DOI: 10.1007/s00277-020-04385-9
Abstrakt: Sickle cell disease (SCD) comprises a group of genetic disorders characterized by the presence of the hemoglobin (Hb) S in homozygosis or in heterozygosis with some other Hb variant or in interaction with thalassemia. SCD is characterized by a very complex pathophysiology, which determines a wide variability of clinical manifestations, including a chronic state of hypercoagulability responsible for the increased risk of thromboembolic events. ADAMTS13 and von Willebrand factor (VWF) play an important role in arterial and venous thrombosis. Thus, the aim of this study was to understand how the ADAMTS13-VWF axis behaves in sickle cell disease, as well as whether there is an association of these markers with the use of hydroxyurea (HU). This is a cross-sectional study conducted with 40 patients diagnosed with SCD and 40 healthy individuals. The analysis of the ADAMTS13-VWF axis was comparatively performed between groups of patients and controls and, afterwards, between patients with SCD who were users and non-users of HU. ADAMTS13 activity, ADAMTS13 activity/VWF:Ag, and ADAMTS13:Ag/VWF:Ag ratios were significantly lower and VWF:Ag levels significantly higher in SCD patients when compared to the controls. There was no statistically significant difference in ADAMTS13:Ag and VWF collagen binding (VWF:CB) levels between the groups evaluated. Among the categories of HU use, there was no statistically significant difference in any of the evaluated markers. As a conclusion, we could observe that the ADAMTS13-VWF axis is altered in SCD when compared to healthy individuals and that there is no association between these markers and the use of HU.
Databáze: MEDLINE
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