Mild sickle-cell anaemia in Iran associated with high levels of fetal haemoglobin.
Sixteen subjects, with sickle-cell anaemia, all Iranians (ages 3 to 56 years), with very mild symptomatology are reported. Some of the subjects had been totally asymptomatic. Splenomegaly was noted in 9 cases. There was an increase in the mean level of fetal haemoglobin (18%); this is the probable explanation for the mild phenotype. In 29 subjects with sickle-cell trait, the level of HbF was also significantly raised as compared with normal (1-6% vs. 0-6%). The mechanism of increased synthesis of HbF is unknown. The findings are similar to those reported in the Shiite Moslems of Saudi Arabia suggesting that in these populations there is a genetically-determined ability to produce high levels of Hb F in the presence of the sickle-cell gene.
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D004906:Erythrocyte Count; D005260:Female; D005319:Fetal Hemoglobin / Q000032:analysis*; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D007492:Iran; D008297:Male; D010641:Phenotype; D012156:Reticulocytes
D006454:Hemoglobins; D005319:Fetal Hemoglobin