Fetal hemoglobin and hemolysis markers in sickle cell anemia

Colella, Marina Pereira; Traina, Fabı́ola

doi:10.1016/j.bjhh.2015.03.015

Cited by 3 publications

(1 citation statement)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…HbF is the major hemoglobin during gestation and constitutes approximately 60%-80% of the total hemoglobin in full-term newborns. 8 It is then almost completely replaced by adult hemoglobin (hemoglobin A, HbA; α 2 β 2 ) by the approximate age of 6-12 months, and it amounts to less than 1% of the total hemoglobin in adults.…”

Section: Introductionmentioning

confidence: 99%

Association of polymorphisms in the HBG1‐HBD intergenic region with HbF levels

Huang

Han

et al. 2020

Clinical Laboratory Analysis

View full text Add to dashboard Cite

Background: Increased levels of fetal hemoglobin (HbF) can improve the clinical course of the patients with sickle cell anemia (SCA) or β-thalassemia. The HBG1-HBD intergenic region plays an important role in this process. However, very few studies investigated whether the variations in this region have an effect on HbF expression. Methods:We retrieved all the SNP data in the HBG1-HBD intergenic region and defined the haplotype blocks, then performed cluster analysis and selected a tagSNP.A total of 500 normal individuals and 300 β-thalassemia carriers were enrolled. After routine blood and hemoglobin capillary electrophoresis testing, β-thalassemia mutations were detected using PCR-reverse dot blot. The genotypes of the rs4910736 (A > C) and rs10128556 (C > T) were determined using Sanger sequencing; the relationship between the two SNPs and the levels of HbF was analyzed.Results: Two haplotype blocks were constructed. Block 1 included seven haplotypes divided into two groups M and N by 11 tagSNPs, among which rs4910736 was selected as a tagSNP, while block 2 included three haplotypes. We found that the haplotypes of block 1 were statistically associated with HbF levels, but the non-tagSNP rs10128556 was shown to be more strongly associated with HbF levels than rs4910736. Conclusion:This work proved that the haplotypes in the HBG1-HBD intergenic region and SNP rs10128556 are both statistically associated with HbF levels, revealing the association of polymorphisms in the HBG1-HBD intergenic region with HbF levels.

show abstract

Section: Introductionmentioning

confidence: 99%

Association of polymorphisms in the HBG1‐HBD intergenic region with HbF levels

Huang

Han

et al. 2020

Clinical Laboratory Analysis

View full text Add to dashboard Cite

show abstract

Red blood cell adhesion to ICAM-1 is mediated by fibrinogen and is associated with right-to-left shunts in sickle cell disease

et al. 2020

View full text Add to dashboard Cite

Sickle cell disease (SCD), which afflicts 100 000 Americans, as well as millions worldwide, is associated with anemia, lifelong morbidity, and early mortality. Abnormal adhesion of sickle red blood cells (RBCs) to activated vascular endothelium may contribute acutely to the initiation of painful vaso-occlusive crises and chronically to endothelial damage in SCD. Sickle RBCs adhere to activated endothelium through several adhesion mechanisms. In this study, using whole blood from 17 people with heterozygous SCD (HbS variant) and 55 people with homozygous SCD (HbSS) analyzed in an in vitro microfluidic assay, we present evidence for the adhesion of sickle RBCs to immobilized recombinant intercellular adhesion molecule 1 (ICAM-1). We show that sickle RBC adhesion to ICAM-1 in vitro is associated with evidence of hemolysis in vivo, marked by elevated lactate dehydrogenase levels, reticulocytosis, and lower fetal hemoglobin levels. Further, RBC adhesion to ICAM-1 correlates with a history of intracardiac or intrapulmonary right-to-left shunts. Studies of potential ICAM-1 ligands on RBC membranes revealed that RBC–ICAM-1 interactions were mediated by fibrinogen bound to the RBC membrane. We describe, for the first time, RBC rolling behavior on ICAM-1 under high shear rates. Our results suggest that firm adhesion of sickle RBCs to ICAM-1 most likely occurs in postcapillary venules at low physiological shear rates, which is facilitated by initial rolling in high shear regions (eg, capillaries). Inhibition of RBC and ICAM-1 interactions may constitute a novel therapeutic target in SCD.

show abstract

The effects of fetal hemoglobin levels on sickle cell anemia patients bone biochemistry / Fetal hemoglobin seviyesinin orak hücre anemisi hastalarının kemik biyokimyası üzerine etkisi

Kökbaş

Yenilmez

Tuli

et al. 2016

Turkish Journal of Biochemistry

View full text Add to dashboard Cite

Objective: The aim of this study was to evaluate the possible roles of fetal hemoglobin levels on bone parameters in sickle cell anemia (SCA) patients.Methods: Blood samples taken from 56 SS and 47 control totally 103 subjects were included in this research work according to their fetal hemoglobin levels. Fetal hemoglobin, bone mineral density, bone specific alkaline phosphatase, calcium, osteocalcin, 25-OH vitamin D and hematological parameters were measured and analyzed in the study.Results: Statistical analysis showed that, lower bone mineral density and biochemical bone parameters significantly correlated with low fetal hemoglobin levels at SCA patients.Conclusion: It was concluded that fetal hemoglobin level is a good index for bone status in sickle cell anemia patients.

show abstract

Fetal hemoglobin and hemolysis markers in sickle cell anemia

Cited by 3 publications

References 11 publications

Association of polymorphisms in the HBG1‐HBD intergenic region with HbF levels

Association of polymorphisms in the HBG1‐HBD intergenic region with HbF levels

Red blood cell adhesion to ICAM-1 is mediated by fibrinogen and is associated with right-to-left shunts in sickle cell disease

The effects of fetal hemoglobin levels on sickle cell anemia patients bone biochemistry / Fetal hemoglobin seviyesinin orak hücre anemisi hastalarının kemik biyokimyası üzerine etkisi

Contact Info

Product

Resources

About