Please use this identifier to cite or link to this item:
|Title:||Coinheritance of the different copy numbers of α-globin gene modifies severity of β-thalassemia/Hb E disease|
The Institute of Science and Technology for Research and Development, Mahidol University
|Citation:||Annals of Hematology. Vol.87, No.5 (2008), 375-379|
|Abstract:||β-Thalassemia/Hb E patients show a range of clinical severities, from nearly asymptomatic to transfusion-dependent thalassemia major. This study investigated the clinical heterogeneity and hematologic parameters obtained in the large cohort of 925 Thai β0-thalassemia/Hb E patients. Coinheritance of α-thalassemia with β0-thalassemia/Hb E produces a milder clinical phenotype in contrast to an interaction of α-globin gene triplication in severe thalassemia. The mean steady-state Hb was also higher, whereas the mean corpuscular volume and the percentage of Hb F were markedly lower in the former group. This finding demonstrates that the genetic combination leading to the more/less degree of α- to non-α-globin chains imbalance is indeed the cause of the severe/ mild thalassemia phenotype. © Springer-Verlag 2007.|
|Appears in Collections:||Scopus 2006-2010|
Files in This Item:
There are no files associated with this item.
Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.