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Cited by 79Hemoglobinopathies and Related DisordersHemoglobin structure and functionNeonatal Health and Biochemistry

Variation in fetal hemoglobin parameters and predicted hemoglobin S polymerization in sickle cell children in the first two years of life: Parisian Prospective Study on Sickle Cell Disease

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Maier-Redelsperger, M, Noguchi, CT, de Montalembert, M, Rodgers, GP, Schechter, AN, Gourbil, A, Blanchard, D, Jais, JP +2 more·1994·Blood
DOI 10.1182/blood.v84.9.3182.3182
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Abstract

Intracellular hemoglobin S (HbS) polymerization is most likely to be the primary determinant of the clinical and biologic manifestations of sickle cell disease (SCD). Fetal hemoglobin (HbF) does not enter the HbS polymer and its intracellular expression in sickle erythrocytes inhibits polymerization. HbF levels, high at birth but decreasing thereafter, protect the newborn from the clinical manifestations of this hemoglobinopathy. We have measured the sequential changes in HbF, F reticulocytes, and F cells in the first 2 years of life in 25 children with SCD and compared the results with those obtained in 30 normal children (AA). We have also calculated HbF per F cell (F/F cell), the preferential survival of F cells versus non-F cells, as measured by the ratio F cells versus F reticulocytes (FC/FR) and polymer tendency at 40% and 70% oxygen saturation. HbF levels decreased from about 80.4% +/- 4.0% at birth to 9.2% +/- 2.9% at 24 months. During this time, we observed a regular decrease of the F reticulocytes and the F cells. The kinetics of the decline of F/F cell was comparable with the decline of HbF, rapid from birth (mean, 27.0 +/- 3.6 pg) to 12 months of age (mean, 8.5 +/- 1.5 pg) and then slower from 12 to 24 months of age (mean, 6.2 +/- 1.0 pg) in the SCD children. In the AA children, the decrease in HbF, due to changes in both numbers of F cells and F/F cell, was more precipitous, reaching steady-state levels by 10 months of age. Calculated values for mean polymer tendency in the F-cell population showed that polymerization should begin to occur at 40% oxygen saturation at about 3 months and increase progressively with age, whereas polymerization at 70% oxygen saturation would not occur until about 24 months. These values correspond to HbF levels of 50.8% +/- 10.8% and 9.2% +/- 2.9%, respectively, and F/F cell levels of 15.6 +/- 4.5 pg and 6.2 +/- 1.0 pg, respectively. In the non--F-cell population, polymerization was expected at birth at both oxygen saturation values. Three individuals had significantly greater predicted polymerization tendency than the remainder of the group because of early decreases in HbF. These individuals in particular, the remainder of the cohort, as well as other recruited newborns, will be studied prospectively to ascertain the relationship among hematologic parameters, which determine polymerization tendency and the various clinical manifestations of SCD.

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  • When HbF/F-cell is 9 to 12 pg, calculations suggested that deoxyHbS polymerization should be prevented at physiologic venous and capillary O2 saturations of 40% to 70%, affording the sickle erythrocyte nearly total protection from HbS polymer-induced damage, although recent studies call this observation into question.

    “When HbF/F-cell is 9 to 12 pg, calculations suggested that deoxyHbS polymerization should be prevented at physiologic venous and capillary O 2 saturations of 40% to 70%, affording the sickle erythrocyte nearly total protection from HbS polymerinduced damage, although recent studies call into question this observation.3,48,49”

    From Fetal hemoglobin in sickle cell anemia (2020) · cites this paper as [48]

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Research works printed but not linked: 2

  1. [11]11. Schechter AN, Noguchi CT, Rodgers GP: Sickle cell disease, in Stamatoyannopoulos G, Nienhuis AW, Leder P, Majerus P (eds): Molecular Basis of Blood Diseases. Philadelphia, PA, Saunders, 1987, p 179
  2. [24]24. Serjeant GR: Sickle Cell Disease (ed 2). Oxford, UK. Oxford. 1992

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Authors
Maier-Redelsperger, M., Noguchi, C., de Montalembert, M., Rodgers, G., Schechter, A., Gourbil, A., Blanchard, D., Jais, J., Ducrocq, R., & Peltier, J.
Affiliations
  • Laboratoire d'Hematologie, Hopital Tenon, Paris, France.
  • Hôpital Tenon (FR)
Venue
Blood
Publisher
American Society of Hematology
Published
1994-11-01
Volume
84
Issue
9
Pages
3182-3188
ISSN
  • 0006-4971 (ISSN-L)
  • 1528-0020
DOI
10.1182/blood.v84.9.3182.3182
OpenAlex
W2343427948
Cited by
79
References
28
Citations by year
2026: 1 · 2025: 3 · 2024: 6 · 2023: 2 · 2022: 6 · 2021: 4 · 2020: 5 · 2019: 3 · 2018: 5 · 2017: 6 · 2016: 3 · 2015: 3 · 2014: 2 · 2013: 3 · 2012: 4
FWCI
1.47
Type
journal article
Language
en
Open access
Locations
  • Blood (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Genetics › Hemoglobinopathies and Related Disorders
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Hemoglobin structure and function
  • Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Neonatal Health and Biochemistry
Keywords
sickle cell disease, fetal hemoglobin, hemoglobin S polymerization, F cells, oxygen saturation, infants, sickle erythrocytes
MeSH terms
Anemia, Sickle Cell, Erythrocytes, Abnormal, Female, Fetal Hemoglobin, Hemoglobin, Sickle, Humans, Infant, Infant, Newborn, Male, Polymers, Prospective Studies, Sex Factors, alpha-Thalassemia

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