Adult and Fetal Hemoglobin Production in Erythroid Colonies from Subjects with β-Thalassemia or with Hereditary Persistence of Fetal Hemoglobin (HPFH)

Abstract

The synthesis of α and non-α chains (β, δ, ^Gγ, and ^Aγ) was studied in cultures of peripheral blood mononuclear cells from eleven β-thalassemia heterozygotes, two HPFH heterozygotes, and one HPFH homozygote. The synthesis of Hb F in the thalassemia colonies (average value: 12.6%) was comparable to that in normal adult colonies (average value: 12.2%). The percent ^Gγ chain in the Hb F varied greatly but a relationship between the ^Gγ chain percentage in the Hb F from colonies and that from peripheral blood was established. The relative synthesis of Hb A₂ in colonies of β-thalasse-mia heterozygotes (average value: 5.8%) was 1.6 times as much as that in colonies of normal adults (average value: 3.6%). Hb A₂ and Hb A were absent in the colonies of the HPFH homozygote.