The use of modern DNA techniques enabled us to characterize and identify 44 distinct beta-thal mutations and nine alpha-thal genotypes in the UAE population. All of the beta-thal mutations were severe beta+ or beta0 types resulting in transfusion-dependent phenotypes. Furthermore, a large number of alphaT alleles in the alpha-thal carriers and in patients with Hb H disease, accentuate the impor...

Some people may be laughing when looking at you reading in your spare time. Some may be admired of you. And some may want be like you who have reading hobby. What about your own feel? Have you felt right? Reading is a need and a hobby at once. This condition is the on that will make you feel that you must read. If you know are looking for the book enPDFd sickle cell anemia and other hemoglobino...

Hemoglobinopathies and other disorders of erythroid cells are often associated with abnormal iron homeostasis. We review the molecular physiology of intracellular and systemic iron regulation, and the interactions between erythropoiesis and iron homeostasis. Finally, we discuss iron disorders that affect erythropoiesis as well as erythroid disorders that cause iron dysregulation.

Characterization of the molecular basis of the β-thalassemias and sickle cell disease (SCD) clearly showed that individuals with the same β-globin genotypes can have extremely diverse clinical severity. Two key modifiers, an innate ability to produce fetal hemoglobin and coinheritance of α-thalassemia, both derived from family and population studies, affect the pathophysiology of both disorders...

Haptoglobin types were determined in 626 individuals living in the State of São Paulo (Brazil). Of these, 484 had Hb AA, 31 major beta thalassemia, 43 minor beta thalassemia, 14 Hb SS, and 54 Hb AS. Frequency distribution of the three most common types observed among patients with type beta thalassemia differed significantly from that observed in the Caucasian group with Hb AA. There was a sign...

In utero hematopoietic cell transplantation (IUHCTx) is a promising strategy to circumvent the challenges of postnatal hematopoietic stem cell (HSC) transplantation. The goal of IUHCTx is to introduce donor cells into a naïve host prior to immune maturation, thereby inducing donor-specific tolerance. Thus, this technique has the potential of avoiding host myeloablative conditioning with cytotox...

Hemoglobinopathies are very common in Greece, the incidence of beta-thalassemia trait being 8% and that of sickle cell trait ranging from 1 to 32% in various districts. In Greek populations, sickle cell disease (SCD) is mainly represented by S-beta thalassemia.