Relationship of haemoglobin F and alpha thalassaemia to severity of sickle-cell anaemia in the Eastern Province of Saudi Arabia.
To define the natural history of sickle cell anaemia a cord blood screening programme was initiated and during the first 3 years a total of 129 newborns with FS phenotype were detected, of whom 83 infants were followed up regularly. Forty-three of them had attained the ages of between 1 and 3 years and the analysis of their data revealed that in 21 of 43 cohort children (14 FS and 7 FS + Barts) who had almost identically raised levels of haemoglobin F, the clinical manifestations of the disease appeared to be influenced by the size of the RBC, with the microcytic group having a relatively milder clinical course as compared with the normocytics. Similar observations were made in about 50% (47/93) of non-cohort older children and adults with sickle-cell anaemia, suggesting that alpha thalassaemia in combination with increased levels of fetal haemoglobin was probably an important predictor of the clinical severity in patients with sickle-cell anaemia in the Eastern Province of Saudi Arabia.
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005312:Fetal Blood / Q000032:analysis; D005319:Fetal Hemoglobin / Q000032:analysis*; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010641:Phenotype; D012529:Saudi Arabia; D013789:Thalassemia / Q000150:complications*
D005319:Fetal Hemoglobin