Beta-thalassemias are a group of hereditary blood disorders characterized by anomalies in the synthesis of the beta chains of hemoglobin resulting in variable phenotypes ranging from severe anemia to clinically asymptomatic individuals. The total annual incidence of symptomatic individuals is estimated at 1 in 100,000 throughout the world and 1 in 10,000 people in the European Union. Three main...

Beta-thalassemia is one of the most prevalent autosomal disorders in the world. Mutations in the HBB gene underlie deficiencies in hemoglobin production, which can interfere with oxygen delivery resulting in wide range of disease severity. Although >535 mutations have been characterized in the HBB gene, beta-thalassemia is broadly classified into three groups, based on clinical severity: beta-t...

Background and aims: Thalassemia is one of the most prevalent hematologic disorders worldwide. Thalassemia is the most common inherited anemia and genetic disease. Diabetes mellitus and insulin resistance is one of the major endocrine problems in major thalassemia patients. This study was done to evaluate the association of serum γinterferon and IL-10 concentrations with insulin resistance in s...

The Sibling Donor Cord Blood (SDCB) Program was initiated in 1998 as a resource to collect, characterize, and release cord blood units (CBUs) from families affected by malignant and nonmalignant disorders for transplantation. Families in the United States were recruited by telephone after referrals by community and academic physicians. Collection kits were mailed to prospective participants and...

Using a mouse monoclonal antibody (mAb) (“2D4”) with high specific reactivity to Hb Bart’s and a rabbit polyclonal antibody (“RPB”) with high reactivity to Hb Bart’s but low reactivity to HbF, an ELISA assay was developed for the quantification of Hb Bart’s in hemolysates of peripheral blood. In the preliminary study, hemoglobin solutions containing 4,000 μg/mL of hemoglobin were analyzed for t...

background: studies have demonstrated that sickle cell trait can be found in an asymptomatic healthy carrier with normal complete blood count (cbc) and red blood cell (rbc) indices. according to iranian ministry of health bulletin instructions, prenuptial thalassemia screening program (tsp) primarily depends on rbc indices which are measured through a routine cbc. only when these levels are bel...

a, fi, and ‘i globin chain synthesis in bone marrow and peripheral blood reticulocytes were studied in two patients with thalassemia major, two with thalassemia intermedia, one with thaIassemia minor, one with Hb H disease, and one with homozygous f38-thalassemia. Nine nonthalassemic patients served as controls. In thalassemia major, a marked imbalance of ato fichain synthesis was found in the ...

BACKGROUND Thalassemia is a heterogeneous disorder and several genetic factors influence the severity of thalassemia. An accurate and early diagnosis of a mild thalassemia genotype helps to avoid unnecessary transfusion and its complications. The aim of this study is to identify the association between XmnI polymorphism and disease severity in patients with beta-thalassemia from northern Pakist...

BACKGROUND & OBJECTIVE Alpha (α) thalassemia is a hereditary disorder and is caused by deletions or mutations in globin genes. It is present in two clinically significant forms: hemoglobin Bart hydrops fetalis (Hb Bart) syndrome and hemoglobin H (HbH) disease. It is highly prevalent in South-East Asia or Mediterranean countries. The most common deletion reported in alpha thalassemia in Pakistan...