| Literature DB >> 18026953 |
Orapan Sripichai1, Thongperm Munkongdee, Chutima Kumkhaek, Saovaros Svasti, Pranee Winichagoon, Suthat Fucharoen.
Abstract
beta-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 beta 0-thalassemia/Hb E patients. Coinheritance of alpha-thalassemia with beta 0-thalassemia/Hb E produces a milder clinical phenotype in contrast to an interaction of alpha-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 alpha- to non-alpha-globin chains imbalance is indeed the cause of the severe/mild thalassemia phenotype.Entities:
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Year: 2007 PMID： 18026953 DOI： 10.1007/s00277-007-0407-2
Source DB: PubMed Journal: Ann Hematol ISSN： 0939-5555 Impact factor: 3.673