Hemoglobin F and hemoglobin E/beta-thalassemia.

作者: D. C. Rees

DOI: 10.1097/00043426-200011000-00025

关键词:

摘要: Hemoglobin (Hb) F levels are high and variable in Hb E/beta-thalassemia, ranging from 10% to 80%. The secondary expansion of the erythron with ineffective erythropoiesis selection favor cells able make more gamma-globin. variability reflects complex processes involved production. Important determinants include age, alpha-thalassemia, genetic gamma-chain synthesis. Overall, percentage correlates total hemoglobin decreases steadily age. alpha-thalassemia is associated a lower but higher level E/beta-thalassemia.

参考文章(15)
D.C. Rees, J.B. Clegg, D.J. Weatherall, Is Hemoglobin Instability Important in the Interaction Between Hemoglobin E and β Thalassemia Blood. ,vol. 92, pp. 2141- 2146 ,(1998) , 10.1182/BLOOD.V92.6.2141
D.C. Rees, J.B. Porter, J.B. Clegg, D.J. Weatherall, Why are hemoglobin F levels increased in HbE/beta thalassemia? Blood. ,vol. 94, pp. 3199- 3204 ,(1999) , 10.1182/BLOOD.V94.9.3199
HA Huebers, Y Beguin, P Pootrakul, D Einspahr, CA Finch, Intact transferrin receptors in human plasma and their relation to erythropoiesis. Blood. ,vol. 75, pp. 102- 107 ,(1990) , 10.1182/BLOOD.V75.1.102.102
GJ Dover, KD Smith, YC Chang, S Purvis, A Mays, DA Meyers, C Sheils, G Serjeant, Fetal hemoglobin levels in sickle cell disease and normal individuals are partially controlled by an X-linked gene located at Xp22.2 Blood. ,vol. 80, pp. 816- 824 ,(1992) , 10.1182/BLOOD.V80.3.816.BLOODJOURNAL803816
R Galanello, S Barella, MP Turco, N Giagu, A Cao, F Dore, NL Liberato, R Guarnone, G Barosi, Serum erythropoietin and erythropoiesis in high- and low-fetal hemoglobin β-thalassemia intermedia patients Blood. ,vol. 83, pp. 561- 565 ,(1994) , 10.1182/BLOOD.V83.2.561.561
Jamie E. Craig, Jacques Rochette, Chris A. Fisher, David J. Weatherall, Sophie Marc, G. Mark Lathrop, Florence Demenais, SweeLay Thein, Dissecting the loci controlling fetal haemoglobin production on chromosomes 11p and 6q by the regressive approach. Nature Genetics. ,vol. 12, pp. 58- 64 ,(1996) , 10.1038/NG0196-58
P. C. Rutland, M. E. Pembrey, T. Davies, The estimation of fetal haemoglobin in healthy adults by radioimmunoassay. British Journal of Haematology. ,vol. 53, pp. 673- 682 ,(1983) , 10.1111/J.1365-2141.1983.TB07319.X
J. Rochette, J.E. Craig, S.L. Thein, J. Rochette, Fetal hemoglobin levels in adults Blood Reviews. ,vol. 8, pp. 213- 224 ,(1994) , 10.1016/0268-960X(94)90109-0
D. C. REES, L. STYLES, E. P. VICHINSKY, J. B. CLEGG, D. J. WEATHERALL, The Hemoglobin E Syndromes Annals of the New York Academy of Sciences. ,vol. 850, pp. 334- 343 ,(1998) , 10.1111/J.1749-6632.1998.TB10490.X
D. J. WEATHERALL, J. B. CLEGG, W. G. WOOD, S. T. CALLENDER, B. L. SHERIDAN, J. PRITCHARD, Foetal erythropoiesis in human leukaemia. Nature. ,vol. 257, pp. 710- 712 ,(1975) , 10.1038/257710A0