Few literatures have elaborated on the clinical characteristics of children with thalassemia from low-prevalence areas. A retrospective analysis was conducted on children genetically confirmed with thalassemia at Seoul National University Children's Hospital in Korea. Nine children (1α thalassemia trait, 6β thalassemia minor, 2β thalassemia intermedia) were diagnosed with thalassemia at median ...

Patients with thalassemia intermedia have a mild anemia and survive without needing regular blood transfusions.1 Here we report two cases of Thai patients with β-thalassemia intermedia caused by homozygosity of hemoglobin Malay (Hb Malay; α2β219Asn-Ser)2 and compound heterozygosity of Hb Malay and hemoglobin E (Hb E; α2β226Glu-Lys). Both patients presented with a history of anemia with marked m...

To investigate the molecular basis of β -thalassemia intermedia in Northern Iraq and evaluate its management practices, a total of 74 patients from 51 families were enrolled. The patients were clinically and hematologically reevaluated, and had their β-thalassemia mutations characterized, as well as the number of α-globin genes and Xmn I (G)γ-158 (C>T) polymorphism studied. Out of 14 β-thalasse...

Beta thalassemia dominant results from mutations in the β globin chain gene resulting in the production of elongated, highly unstable beta globin chains. Several such mutations have been described and in a heterozygous state they may confer a phenotype more severe than that of β thalassemia trait and lead to a clinical syndrome of thalassemia intermedia and its associated complications such as ...

Pulmonary hypertension (PH) is frequent among patients with β-thalassemia intermedia (TI) and β-thalassemia major (TM) (1). Almost 60% of all TI patients develop PH (2). However, no randomized controlled trials have evaluated this condition-specific treatment options. Recent guidelines for the treatment of PH offer no specific recommendations for these patients; moreover, the classification of ...

BACKGROUND Leishmania transmission occurs in the presence of insect saliva. Immunity to Phlebotomus papatasi or Lutzomyia longipalpis saliva or salivary components confers protection against an infection by Leishmania in the presence of the homologous saliva. However, immunization with Lutzomyia intermedia saliva did not protect mice against Leishmania braziliensis plus Lu. intermedia saliva. I...

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...

hydroxyurea (hu) has been successfully used in patients with β-thalassemia intermedia (β-ti). we aimed to evaluate the effect of the long-term use of hu on thyroid function in patients with β-ti. seventy-five patients with β-ti aged≥11 years and taking hu were randomly selected during 2010 in southern iran. thirty-one patients with β-ti without hu were considered as a control group. serum level...

Extramedullary hematopoiesis (EMH), as a compensatory phenomenon, refers to the blood cell formation outside of bone marrow that occurs once cells in circulatory system fail meet individuals’ needs. EMH is rare moderate severe beta thalassemia because most symptomatic patients are effectively managed with transfusion. However, receive transfusions like β-thalassemia intermedia (β-TI) indicated ...

Non-transfusion-dependent thalassemias include a variety of phenotypes that, unlike patients with beta (β)-thalassemia major, do not require regular transfusion therapy for survival. The most commonly investigated forms are β-thalassemia intermedia, hemoglobin E/β-thalassemia, and α-thalassemia intermedia (hemoglobin H disease). However, transfusion-independence in such patients is not without ...