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...

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. ...

The management of patients with non-transfusion-dependent thalassaemia (NTDT) has been a challenging task: in fact, within this conventional definition, clinicians have to deal with a great variety of syndromes mixed in terms of their molecular background, clinical course, and severity which share only the characteristics that are not entirely dependent on transfusions [1, 2]. In fact, NTDT phe...

Introduction: Beta-thalassemia is an autosomal recessive disease that occurs as a result of disorder in the (β-globin chains synthesis), and gold standard method for diagnosis genetic mutation analysis. It important to know distribution mutations according regions races. The aim this study document beta-globin gene beta-thalassemia major intermedia patients who were followed treated Sanliurfa p...

background : using two logistic regression models, we determined the associates of poor physical and mental health related quality of life (hrqol) among beta thalassemia patients. methods : in this cross-sectional study which was conducted during 2006 and 2007 in outpatient adult thalassemia clinic, blood transfusion organization, tehran, iran, short form 36 (sf-36) was used for measuring hrqol...

Hydroxyurea (HU) enhances fetal hemoglobin (Hb) production. An increase in total Hb level has been repeatedly reported during HU treatment in patients with sickle cell disease and in several patients with beta-thalassemia intermedia. Effects in patients with beta-thalassemia major are controversial. We now report a marked elevation of total Hb levels with HU that permitted regular transfusions ...