Hb F in sickle cell anemia

Experientia. 1993 Jan 15;49(1):16-27. doi: 10.1007/BF01928784.

Abstract

We have reviewed the methodology for an accurate quantitation of Hb F in the blood of patients with sickle cell anemia, values observed in hundreds of patients of different (racial or ethnic) backgrounds and with differences in severity of the disease, and the various factors that affect the level of Hb F. The latter include sex, age, genetic background or chromosomal haplotypes, variations in the sequences of the locus control region(s) 5' to the epsilon-globin gene, and the presence of an alpha chain deficiency or alpha-thalassemia. Finally, a few remarks about agents effective in increasing the in vivo Hb F synthesis are also included.

Publication types

  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Anemia, Sickle Cell / blood*
  • Child
  • Child, Preschool
  • Female
  • Fetal Hemoglobin / analysis*
  • Fetal Hemoglobin / biosynthesis
  • Globins / genetics
  • Haplotypes
  • Hemoglobin, Sickle / analysis
  • Humans
  • Male
  • Mutation
  • Sex Factors

Substances

  • Hemoglobin, Sickle
  • Globins
  • Fetal Hemoglobin