Hemoglobin E/β-thalassemia (HbE/β-thalassemia) is the most important type of thalassemia in northeastern Thailand. Serious complications of the disease are associated with iron overload and the consequences of oxidative damage to various organs, especially the cardiovascular system. Endothelial dysfunction is an important predictor for the long-term outcome of the disease. In this study, 19 pat...

In sickle cell disease (SCD), hydroxyurea (HU) treatment decreases the number of vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) by increasing fetal hemoglobin (HbF). Data are lacking regarding the frequency of HU dose modification or whether sub-therapeutic doses (<15 mg/kg/day) are beneficial. We reviewed the medical records of 140 patients from 2010 to 2014. The laboratory paramet...

by application of modern recombinant dna technology, especially the polymerase chain reaction (pcr)/dot-blot hybridization techniques, we have investigated the molecular basis of β-thalassemia from four different regions of iran: central, south-east, south and north. in this study, the dna samples were isolated from patients and for the identification of the mutations, the 6 oligonucleotide pro...

OBJECTIVE The diverse clinical phenotype of hemoglobin E (HbE)/β-thalassemia has not only confounded clinicians in matters of patient management but has also led scientists to investigate the complex mechanisms involved in maintaining the delicate red cell environment where, even with apparent similarities of α- and β-globin genotypes, the phenotype tells a different story. The BTB and CNC homo...

The β-thalassemias are a group of hereditary hematological diseases caused by over 300 mutations of the adult β-globin gene. Together with sickle cell anemia, thalassemia syndromes are among the most impactful diseases in developing countries, in which the lack of genetic counseling and prenatal diagnosis have contributed to the maintenance of a very high frequency of these genetic diseases in ...

Introduction: Beta-thalassemia is characterized by absence or reduced synthesis of the β-globin. Carriers of β-thalassemia, typically have microcytic hypochromic anemia and elevated hemoglobin HbA2 and normal HbF level. On the other hand carriers of severe alpha-thalassemia also have similar CBC parameters to that of β-thalassemia with normal HbA2 level. Co-presence of mutations in the β-globin...

conclusions: hydroxyurea can be safely used in some transfusion-dependent β-thalassemia patients to decrease their transfusion needs. background: β-thalassemia is an inherited hemoglobin disorder caused by defective synthesis of ß-globin chains. hemoglobin (hb) f induction is a possible therapeutic approach which can partially compensate for α and non-α globin chains imbalance. objectives: we a...

Background: Transfusion is the mainstay treatment of patients with thalassemia major and occasionally in thalassemia intermediate. Alloimmunization is an unwanted side effect of blood transfusion. The present study intended to determine the frequency of alloimmunization in patients with β- thalassemia major and thalassemia intermediate in Southwest Iran. Patients and Methods: This was a cross-...

background: transfusion is the mainstay treatment of patients with thalassemia major and occasionally in thalassemia intermediate. alloimmunization is an unwanted side effect of blood transfusion. the present study intended to determine the frequency of alloimmunization in patients with β- thalassemia major and thalassemia intermediate in southwest iran. patients and methods: this was a cross-s...

Introduction. Patients with β-thalassemia intermedia have a higher incidence of thromboembolic events compared to the general population. Previous studies have shown that patients with sickle cell disease, who are also prone to ischemic events, have higher intracranial arterial blood flow velocities measured by transcranial Doppler sonography (TCD). The aim of this study is to evaluate intracra...