Hemoglobin F mitigation of sickle cell complications decreases with aging

Tolu, Seda; Reyes‐Gil, Morayma; Ogu, Ugochi Olivia; Thomas, Merin; Bouhassira, Eric E.; Minniti, Caterina P.

doi:10.1002/ajh.25759

Cited by 5 publications

(2 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…These results were strengthened by performing in vitro sickling assays using samples from other patients, which was also correlated with the threshold of 4, 6, 8 and 10 pg of HbF/RBC in a stronger way than %HbF and F cell frequency as observed by ROC analysis. While studied on a small cohort, the distribution of HbF (ie, pancellular or heterocellular), which is of interest from a biological point of view, seems to be associated with the VOC incidence, with heterocellular distribution having a negative effect, as reported 37 …”

Section: Discussionmentioning

confidence: 86%

Individual red blood cell fetal hemoglobin quantification allows to determine protective thresholds in sickle cell disease

et al. 2020

View full text Add to dashboard Cite

Polymerization of the sickle hemoglobin (HbS) is a key determinant of sickle cell disease (SCD), an inherited blood disorder. Fetal hemoglobin (HbF) is a major modulator of the disease severity by both decreasing HbS intracellular concentration and inhibiting its polymerization. However, heterocellular distribution of HbF is common in SCD. For HbS polymerization inhibition, the hypothesis of an "HbF per red blood cell (HbF/RBC) threshold" requires accurate measurement of HbF in individual RBC. To date, HbF detection methods are limited to a qualitative measurement of RBC populations containing HbF-the F cells, which are variable. We developed

show abstract

Section: Discussionmentioning

confidence: 86%

Individual red blood cell fetal hemoglobin quantification allows to determine protective thresholds in sickle cell disease

et al. 2020

View full text Add to dashboard Cite

show abstract

“…The MCV of 89.4 fL was unusually high for the HbS-HPFH genotype. The molecular basis of HPFH was not described in detail [21]. Two patients with HbS-HPFH had a history of pain episodes and transfusion.…”

Section: Hbs-gene Deletion Hpfh and δβ Thalassemiamentioning

confidence: 99%

Fetal Hemoglobin in Sickle Hemoglobinopathies: High HbF Genotypes and Phenotypes

Steinberg

2020

JCM

View full text Add to dashboard Cite

Fetal hemoglobin (HbF) usually consists of 4 to 10% of total hemoglobin in adults of African descent with sickle cell anemia. Rarely, their HbF levels reach more than 30%. High HbF levels are sometimes a result of β-globin gene deletions or point mutations in the promoters of the HbF genes. Collectively, the phenotype caused by these mutations is called hereditary persistence of fetal hemoglobin, or HPFH. The pancellularity of HbF associated with these mutations inhibits sickle hemoglobin polymerization in most sickle erythrocytes so that these patients usually have inconsequential hemolysis and few, if any, vasoocclusive complications. Unusually high HbF can also be associated with variants of the major repressors of the HbF genes, BCL11A and MYB. Perhaps most often, we lack an explanation for very high HbF levels in sickle cell anemia.

show abstract

Hydroxyurea and fetal hemoglobin effect on leg ulcers in patients with sickle cell disease

et al. 2022

View full text Add to dashboard Cite

Hemoglobin F mitigation of sickle cell complications decreases with aging

Cited by 5 publications

References 7 publications

Individual red blood cell fetal hemoglobin quantification allows to determine protective thresholds in sickle cell disease

Individual red blood cell fetal hemoglobin quantification allows to determine protective thresholds in sickle cell disease

Fetal Hemoglobin in Sickle Hemoglobinopathies: High HbF Genotypes and Phenotypes

Hydroxyurea and fetal hemoglobin effect on leg ulcers in patients with sickle cell disease

Contact Info

Product

Resources

About