Sickle cell--betao thalassemia variant with high hemoglobin F and mild clinical course.
A 70 year old Black woman had chronic hemolytic anemia without recurrent painful crises. Hemoglobin pattern by electrophoresis was hemoglobin S (69 to 71 per cent), hemoglobin A2 (4.6 per cent) and hemoglobin F (24 to 27 per cent). No hemoglobin A was detected, and the hemoglobin F was distributed heterogeneously in the red cells. Reticulocyte alpha/nonalpha globin chain synthetic ratios were 1.44 to 1.62. Thus, the patient had a high hemoglobin F variant of S-beta zero (betao) thalassemia which has not been described previously. Her clinical course has been mild in comparison with S-betao thalassemia patients who do not have extremely elevated hemoglobin F levels.
D000368:Aged; D000755:Anemia, Sickle Cell / Q000097:blood*; D005260:Female; D005319:Fetal Hemoglobin; D006801:Humans; D013789:Thalassemia / Q000097:blood*
D005319:Fetal Hemoglobin