Abstract
Hypoxia and hemoglobin S polymerization are two triggers responsible for initiating erythrocyte sickling and the consequent clinical sickle cell anemia (SCA) events. The objective of this study was to investigate the expression of hypoxia-responsive genes in SCA, testing for correlation with the clinical-laboratorial characteristics of the patient and hydroxyurea therapy. Our results showed, for the first time, a significantly increased expression of HIF-1α and VEGF genes in patients with SCA and an inverse dose-response relationship with hydroxyurea therapy. These results suggest that hypoxic stress may be involved in both the severity of SCA and its response to treatment.
Keywords:
HIF-1α; VEGF; hydroxycarbamide; hypoxia; sickle cell anaemia.
© 2020 British Society for Haematology and John Wiley & Sons Ltd.
MeSH terms
-
Adolescent
-
Adult
-
Aged
-
Anemia, Sickle Cell / blood
-
Anemia, Sickle Cell / drug therapy
-
Anemia, Sickle Cell / metabolism
-
Anemia, Sickle Cell / pathology
-
Antisickling Agents / therapeutic use
-
Case-Control Studies
-
Female
-
Gene Expression / drug effects
-
Humans
-
Hydroxyurea / therapeutic use*
-
Hypoxia / blood
-
Hypoxia / genetics*
-
Hypoxia / metabolism
-
Hypoxia-Inducible Factor 1, alpha Subunit / biosynthesis
-
Hypoxia-Inducible Factor 1, alpha Subunit / genetics*
-
Male
-
Middle Aged
-
RNA, Messenger / genetics
-
RNA, Messenger / metabolism
-
Vascular Endothelial Growth Factor A / biosynthesis
-
Vascular Endothelial Growth Factor A / genetics*
-
Young Adult
Substances
-
Antisickling Agents
-
HIF1A protein, human
-
Hypoxia-Inducible Factor 1, alpha Subunit
-
RNA, Messenger
-
VEGFA protein, human
-
Vascular Endothelial Growth Factor A
-
Hydroxyurea