Hemoglobin F and hemoglobin E/beta-thalassemia

Authors
Citation
Dc. Rees, Hemoglobin F and hemoglobin E/beta-thalassemia, J PED H ONC, 22(6), 2000, pp. 567-572
Citations number
21
Categorie Soggetti
Pediatrics
Journal title
JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
ISSN journal
10774114 → ACNP
Volume
22
Issue
6
Year of publication
2000
Pages
567 - 572
Database
ISI
SICI code
1077-4114(200011/12)22:6<567:HFAHE>2.0.ZU;2-1
Abstract
Hemoglobin (Hb) F levels are high and variable in Hb E/beta -thalassemia, r anging from 10% to 80%. The high levels are secondary to expansion of the e rythron with ineffective erythropoiesis and selection in favor of cells abl e to make more gamma -globin. The variability in levels reflects the comple x processes involved in Hb F production. Important determinants include age , alpha -thalassemia, and genetic determinants of gamma -chain synthesis. O verall, percentage of Hb F correlates with total hemoglobin levels and decr eases steadily with age, alpha -thalassemia is associated with a lower perc entage of Hb F levels but a higher total hemoglobin level in Hb E/beta -tha lassemia.