Advances in the care of patients with thalassemia, sickle cell disease, and other hemolytic disorders through early detection, education, improvements in red cell transfusion and iron chelation therapy, penicillin prophylaxis, vaccination, and hydroxyurea therapy have led to a significant prolongation of the life expectancy of such patients. As this patient population ages, new chronic complica...

To determine whether beta-thalassemia can be detected in the fetus, blood was obtained from abortuses of normal mothers and of mothers with beta-thalassemia trait. The red cells were incubated with radioactive leucine and the globin chains were analyzed by radiochromatography. Two independent methods were utilized to correct the results for contamination by maternal radioactive beta-chain, and ...

Patients with beta-thalassemia may present with an acquired diffuse elastic tissue defect due to degeneration of elastic tissue along with vaso-occlusive findings in the retinal microvasculature. Here we report the case of a patient with granular-like accumulation presenting as black sunburst lesions detected by optical coherence tomography (OCT). A 38-year-old man with beta-thalassemia interme...

background and purpose: patients with non-transfusion-dependent beta thalassemia major (ntdtm) could reach old age, marry and have children with appropriate care. this study aimed to review the marital status and maternal-fetal outcomes of ntdtm patients at thalassemia research center (trc) of sari, iran. methods: in this study, medical records of patients with β-thalassemia major were reviewed...

Gene transfer for β-thalassemia requires gene transfer into hematopoietic stem cells using integrating vectors that direct regulated expression of β globin at therapeutic levels. Among integrating vectors, oncoretroviral vectors carrying the human βglobin gene and portions of the locus control region (LCR) have suffered from problems of vector instability, low titers and variable expression. In...

Hemoglobin E β thalassemia is the commonest form of severe thalassemia in many Asian countries. Its remarkably variable clinical phenotype presents a major challenge to determining its most appropriate management. In particular, it is not clear why some patients with this condition can develop and function well at very low hemoglobin levels. Here, we demonstrate that patients with hemoglobin Eβ...