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Hb F in sickle cell anemia

1993; Birkhäuser; Volume: 49; Issue: 1 Linguagem: Inglês

10.1007/bf01928784

0014-4754

Adekunle Adekile, T. H. J. Huisman,

Erythrocyte Function and Pathophysiology

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 ε-globin gene, and the presence of an α chain deficiency or α-thalassemia. Finally, a few remarks about agents effective in increasing the in vivo Hb F synthesis are also included.