Background: Effective management of iron overload in patients receiving long-term blood transfusion requires assessment and monitoring of both severity of iron overload and excessive iron chelation. We aimed to evaluate the efficacy and safety of Deferasirox (DFX) in chelation naive patients with transfusion dependent thalassemia and sickle cell disease. Methods: Chelation naive patients with ...

Cardiac disease is the main cause of death in both forms of thalassemia; thalassemia major (TM) and thalassemia intermedia (TI). Pulmonary hypertension (PH) is one of the cardiopulmonary morbidities with high mortality that, if not treated, may trigger right-sided heart failure and premature death. PH is defined as a mean pulmonary artery pressure of ≥25 mmHg at rest or ≥30 mmHg during exercise...

Transfusion dependent congenital sideroblastic anaemia occurred in infancy in two unrelated girls. One girl developed early organ failure which was not prevented by standard chelation treatment. The combination of modest iron burden and putative intrinsic mitochondrial dysfunction could have accounted for the clinical picture. The other girl remained well, receiving regular transfusion and stan...

BACKGROUND The purpose of this study was to evaluate the clinical outcomes of transfusion-associated iron overload in patients with chronic refractory anemia. METHODS Clinical manifestations, main organ function, results of computed tomography (CT), endocrine evaluation, and serum ferritin levels were analyzed retrospectively in 13 patients who were transfusion-dependent for more than 1 year ...

Divergent views remain regarding the safety of treating anemia with red blood cell (RBC) transfusion in patients with acute coronary syndrome (ACS). We used a prospective database to study effect of RBC transfusion in patients with acute myocardial infarction (MI; n = 2,358). Cox regression models were used to determine the association between RBC transfusion and 6-month outcomes, incorporating...

Introduction: The thalassamias refer to a diverse group of hemoglobin disorders characterized by a reduced synthesis of  one or more of globin chains (α,β, γ, δβ,γδβ, δ and εγδβ).The main cure available today for thalassamia is bone marrow transplantation (BMT)  from compatible donor.On December 3, 1981 a 14-month-old child with β-thalassemia major recieved BMT from his HLA-identical sister in...

Background: β-thalassemia is an autosomal hemoglobinopathy with inconsistent universal distribution. Among patients with thalassemia diverse non-siderotic complications distinctly influence the attribute of life, including zinc deficiency due to varied etiologies. The objective of the present study was to determine zinc levels in patients with β-thalassemia major and its correlation with mat...

Background: Beta thalassemia is a common inherited disease, resulting from one or more of 200 different mutations in the beta-globin gene. Qazvin province has attracted migrations of several different populations due to industrialization during the past five decades. The aim of this study was to define the molecular spectrum of beta-thalassemia mutations in Qazvin province. Methods: Ethylen dia...