U NTIL comparatively recently thalassemia was thought to be confined predominantly to persons of Mediterranean origin. it is now abundantly clear that it has a world-wide distribution1 being particularly prevalent in the Far East.2 Reports from Africa ,4 point to the possibility that thalassemia may occur with considerable frequency in the Negro. In addition there is strong evidence that thalas...

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...

BACKGROUND Thalassemia major is one of the most common genetic disorders in Pakistan and over five thousand new patients are added in the pool annually. This familial disease has both medical and social implications, and therefore there is a need to assess the magnitude of beta-Thalassemia trait amongst family members of Thalassemia major patients. METHODS This cross-sectional descriptive stu...

Cation exchange-high performance liquid chromatography (CE-HPLC) is increasingly being used as a first line of investigation for hemoglobinopathies and thalassemias. Together with a complete blood count, the CE-HPLC is effective in categorizing hemoglobinopathies as traits, homozygous disorders and compound heterozygous disorders. We carried out a one year study in Apollo Hospitals, Chennai (Ta...

By PARK S. GERALD AND Louis K. DIAMOND T HIS REPORT details our experiences with a new hemoglobinopathy which we have named the “Lepore trait.” This condition was discovered during a recent survey of the relatives of thalassemia major children.1 The mother of a child with clinical thalassemia major was found to possess a new abnormal hemoglobin (which we have named the Lepore hemoglobin) demons...

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...