Thalassemia is the most common form of all inherited disorders of the red cell. It is estimated that 70 000 children are bornwith various forms of thalassemia each year, andmore than half of these births are affected by severe formsofb-thalassemia, ofwhich themost common subgroup is hemoglobin (Hb) E b-thalassemia. Thalassemia was originally confined to the tropical and subtropical regions of t...

BACKGROUND In sickle cell disease, the quantification of Hb A2 is important for the differential diagnosis between sickle cell anemia (Hb SS) and Hb S/β(0)-thalassemia. OBJECTIVE To determine Hb A2 levels as quantified by high performance liquid chromatography in patients with sickle cell anemia (Hb SS) and with the SC hemoglobinopathy, with or without concomitant alpha thalassemia. METHODS...

BACKGROUND Interpreting the erythroid lineage in populations with high frequency of α+ thalassemia allele is challenging due to the high prevalence of α+ thalassemia homozygotes. For such populations, separate reference values for normal and α+ thalassemia homozygotes are needed. METHODS We studied the erythroid lineage in 1,079 citizens of United Arab Emirates (UAE). Subjects with abnormal h...

β-thalassemia, a congenital genetic hematological disorder characterized by the decrease or absence of β-globin chains, leads to in levels Hemoglobin A. The affected individuals can be categorized into two cohorts based on transfusion dependency: transfusion-dependent thalassemia (TDT) and non-transfusion-dependent (NTDT). Remarkably, despite primary pathology lying chain depletion, β-thalassem...

This study aimed to determine differences in the rates of growth, endocrine- and calcium-related abnormalities in the various thalassemia syndromes in North America treated with current therapies. Medical history, physical examinations and blood and urine collections were obtained from patients with all thalassemia syndromes age 6 years and older in the Thalassemia Clinical Research Network. 36...

OBJECTIVE To describe the challenges, including sociocultural and socioeconomic barriers, faced by an urban immigrant population in the United States affected by thalassemia major. DESIGN Ethnographic, semi-structured, 1-on-1 interviews using an interview guide developed for this study. Digital recordings were transcribed and data analyzed using constant comparative method. SETTING Universi...

Background and Aim: Beta-thalassemia ( -thalassemia) is characterized by the reduced synthesis of the hemoglobin beta chain. Nowadays, more than 200 disease-causing mutations in beta-globin ( -globin) gene have been identified. Betathalassemia is the most common monogenic disease worldwide and one of the widespread hereditary disorders in Iran. Considering the vast spectrum of beta-thalassemia ...

Background: Persons with -thalassemia minor usually are symptomless. However, we previously reported renal ubular dysfunction in a patient with -thalassemia minor. The aim of this study is to investigate renal function in atients with -thalassemia minor. Methods: Forty-one subjects with -thalassemia minor and 20 sexand ge-matched healthy subjects were enrolled in the study. For analysis, patien...

Background Thalassemia is a common hereditary anemia in humans, and beta thalassemia represents a group of recessively inherited hemoglobin disorders first described by Cooley and Lee and characterized by the abnormal synthesis of β-globin chain. The homozygous state results in severe anemia, which needs regular blood transfusion. Although such treatments increase the patient's life span, a var...

From Volume 1 (2011) to 11 (2021), Thalassemia Reports [...]