Hb F Synthesis in Sickle Cell Anaemia: a Comparison of Saudi Arab Cases with those of African Origin |
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| Authors: | W G Wood M E Pembrey G R Serjeant R P Perrine D J Weatherall |
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| Institution: | MRC Molecular Haematology Unit, Nuffield Department of Clinical Medicine, John Radcliffe Hospital, Oxford;Paediatric Genetics Unit, Institute of Child Health, University of London;MRC Laboratories, University of the West Indies, Kingston, Jamaica;Internal Medicine Specialty, Medical Department, ARAMCO, Dhahran, Saudi Arabia |
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| Abstract: | Fetal haemoglobin (Hb F) synthesis has been studied in 22 cases of sickle cell anemia (SS) from Saudi Arabia and compared with an equal number of cases of African origin. Among the Saudi Arabs gamma chain synthesis ranged from 4.0% to 19.9% of the total non-alpha chain synthesis (mean 8.1%) while the corresponding range for the Negro cases was < 0.3% to 4.6% (mean 1.7%). In both groups the peripheral blood Hb F level was on average 3--4 times higher than the proportion synthesized, indicating that the selective survival of Hb F containing cells (F cells) was an important factor in determining the final Hb F levels. Among the Saudi Arab cases there was a significant negative correlation between the degree of F cell enrichment and either the Hb F level of the percentage gamma chain synthesis. No such correlation was observed among the Negro cases. A high proportion of the cases in both groups were carriers of alpha thalassaemia in addition to SS, but no effect of alpha thalassaemia on Hb F production was observed. |
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