A new alkali-resistant hemoglobin alpha2J Oxford gammaF2 in a Sicilian baby girl with homozygous beta0 thalassemia

Blood. 1976 Nov;48(5):639-51.


A 10-mo-old baby girl with homozygous beta0 thalassemia and alphaJOxford, presenting the clinical picture of homozygous beta thalassemia is described. Hemoglobin electrophoresis showed three bands: the first two with the mobilities of hemoglobin Hb A2 (1%) and Hb F (69%), respectively, the third migrating a little faster than Hb A (30%). About 30% of her alpha chains were J Oxford which, bound to her gamma chains, produced a new alkali-resistant hemoglobin, alpha2 J Oxford gamma F2, which has not been described previously. Hemoglobin synthesis in vitro showed the absence of beta chain synthesis and an alpha/non-alpha ratio of 2. The patient's father was heterozygous for both the Hb J Oxford and beta0 thalassemia genes, the mother a carrier of beta0 thalassemia; four other relatives were carriers of Hb J Oxford, and one was a carrier of beta thalassemia.

Publication types

  • Case Reports

MeSH terms

  • Alkalies*
  • Electrophoresis, Cellulose Acetate
  • England
  • Female
  • Globins / biosynthesis
  • Hemoglobin J / analysis*
  • Hemoglobins, Abnormal / analysis*
  • Homozygote*
  • Humans
  • Hydrogen-Ion Concentration
  • Infant, Newborn
  • Pedigree
  • Sicily
  • Thalassemia / blood
  • Thalassemia / genetics*


  • Alkalies
  • Hemoglobins, Abnormal
  • Hemoglobin J
  • Globins