The presence and quantity of hemoglobins Barts and A2 in hemolysates from normal donors and individuals with alpha- and beta-thalassemia trait, respectively, were determined with an enzyme-labeled immunosorbent assay (ELISA). This technique requires the incorporation of monospecific antisera capable of specifically reacting only with these hemoglobins, e.g., with the delta chain of Hb A2 and ga...

Extramedullary hematopoiesis (EMH) is a compensatory physiological proliferation of hematopoietic elements outside the bone marrow as a result of insufficient bone marrow erythropoiesis. It is found in various hematologic disorders (e.g., as sickle cell anemia, thalassemia, myelofibrosis) especially those with chronic anemias. It can affect in any organs, thus producing numerous complications. ...

Background: Hemoglobinopathies are the commonest single gene disorder in human that affect hemoglobin production and function that occur when mutations alter the amino acid sequence of globin chains. The purpose of the present study was to evaluate the prevalence of hemoglobninopathies detected by capillary electrophoresis method in individuals referred to Masjed-Soleiman health centers by capi...

OBJECTIVE Thalassemia is one of the genetic diseases that cause anemia and ineffective erythropoiesis. Increased levels of several inflammatory cytokines have been reported in β-thalassemia and might contribute to ineffective erythropoiesis. However, the mechanism by which tumor necrosis factor-alpha (TNF-α) is involved in ineffective erythropoiesis in thalassemic patients remains unclear. The ...

A flow injection (FI)-reduced volume column system was developed for hemoglobin (Hb) typing to be used as an initial screening method for thalassemia. The column was packed with 140 microl diethylaminoethyl (DEAE)-Sephadex A-50 ion exchange beads. Hb can be separated using Tris-HCl buffer solution with pH gradient 8.5-6.5 and then monitored spectrophotometrically at 415 nm. The hemolysate of 40...

Background: It is customary in Southeast Asia to treat pregnant anemic women with iron supplements, but anemia in this region may be complicated by thalassemia and hemoglobinopathies, which lead to an ineffective response. Objective: The aim was to determine whether routine iron supplementation during pregnancy in this area, which has a high prevalence of thalassemia and hemoglobinopathies, is ...

Despite the vast heterogeneity of mutations, the levels of increased hemoglobin (Hb) A2 seen in individuals of different racial groups heterozygous for the different P-thalassemia mutations are remarkably uniform and rarely more than 6%. However, unusually high levels of Hb A2 have been observed in some P-thalassemia heterozygotes; in one group, it is associated with a partial or complete delet...

BACKGROUND In the past two decades, a large-scale survey of hemoglobinopathies and thalassemia was carried out in mainland China, involving nearly one million people in 28 provinces. The incidences of hemoglobin (Hb) variants, alpha-thalassemia and beta-thalassemia were 0.33%, 2.64% and 0.66%, respectively. The chemical structural analysis identified 67 Hb variants. Among them, 20 are new varia...

The presence of various substitutions and deletions resulting in beta-thalassemia was studied in 19 black patients with homozygous beta-thalassemia and in numerous relatives; all patients were from Georgia, South Carolina, and Alabama. Methodology included gene mapping, amplification of genomic DNA with Taq polymerase, identification of known nucleotide substitutions or a single nucleotide dele...

BACKGROUND Cells respond to stress stimuli through a number of response pathways, of which one of the most important and well characterized is the unfolded protein response. Despite a large body of work which suggests that stress in erythroblasts may play a pivotal role in the pathogenesis of beta-thalassemia/Hb E disease, this pathway remains uninvestigated. DESIGN AND METHODS Day 10 erythro...