Hemoglobin E (HbE; alpha2beta226glu-lys), globally the commonest hemoglobin variant, is synthesized at a slightly reduced rate and has a homozygous phenotype similar to heterozygous beta thalassemia. Yet, when it is inherited together with a beta thalassemia allele, the resulting condition, HbE/beta thalassemia, is sometimes characterized by a severe, transfusion-dependent thalassemia major. Th...

Background: Beta thalassemia is a common health problem in Iran especially in Northern provinces. Premarital screening for thalassemia is compulsory in Iran and identification of the carriers is based on primary CBC (Cell Blood Count) and hemoglobin electrophoresis. Silent mutations on β-globin gene have borderline or normal hematological indices that cannot be detected in premarital scree...

In Southeast Asia alpha-thalassemia, beta-thalassemia, hemoglobin (Hb) E and Hb Constant Spring are prevalent. The gene frequencies of alpha-thalassemia reach 30-40% in Northern Thailand and Laos. beta-Thalassemia gene frequencies vary between 1 and 9%. Hb E is the hallmark of Southeast Asia attaining a frequency of 50-60% at the junction of Thailand, Laos, and Cambodia. Hb Constant Spring gene...

Aim: To assess the prevalence of hepatitis C virus among patients beta-thalassemia. Methods: We enrolled 200 beta-thalassemia in this observational cross sectional study conducted at Department Pathology, Jinnah Medical College/District Specialist Naseerullah Babar Memorial Hospital, Peshawar duration from March, 2022 to August, 2022. determined frequency HCV and assessed association with age g...

Hemoglobinopathy refers to a disease involving qualitative or quantitative defect of the structure synthesis haemoglobin molecules. The HaemoglobinS- beta thalassemia occurs in heterozygotes individual with beta-thalassemia and HaemoglobinS gene. A 29-year-old man came severe anemia, thrombocytopenia, history repeated blood transfusions. Physical examination showed pale conjunctiva, pansystolic...

background and objectives: beta-thalassemia continues to be a cause of significant burden to the society particularly in the poorer developing countries. although sophisticated methods of screening have become available, a hunt for a cheap, rapid, objective screening method still remains elusive. thus, the objectives are to study the validity of naked-eye-single-tube-osmotic-fragility-test (nes...

Homozygous thalassemia is due to inherited unbalanced synthesis of the alpha- or beta-chains of hemoglobin. Clinical severity may be in part related to the extent of alpha:beta imbalance. Two families are presented that illustrate this concept. Thalassemia in these individuals was evaluated by clinical and genetic criteria. The relative rates of alpha- and beta-chain synthesis in their reticulo...

BACKGROUND Doppler echocardiographic studies in patients with beta-Thalassemia Major (beta-TM) had shown different patterns of left ventricle (LV) systolic and diastolic dysfunctions. AIM This cross-sectional study was designed to study the LV systolic and diastolic function in patients with beta-TM using Pulsed Doppler (PD) Echocardiogram and assess the QTc interval and QT dispersion (QTd) o...

Oxidative damage induced by free globin chains has been implicated in the pathogenesis of the membrane abnormalities observed in alpha and beta thalassemia. We have evaluated transport of Na+ and K+ in erythrocytes of patients with thalassemias as well as in two experimental models that use normal human red blood cells, one for alpha thalassemia (methylhydrazine treatment, alpha thalassemia lik...