Thalassemia Intermedia

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Thalassemia intermedia.

Thalassemia intermedia is a clinical definition applied to patients whose clinical phenotype is milder than that of thalassemia major. Criteria used to define thalassemia intermedia including age at presentation, hemoglobin or fetal hemoglobin levels and transfusion independence, are unsatisfactory. The possibility of typing the molecular defect allows a retrospective analysis of patients and o...

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Thalassemia intermedia: revisited.

Thalassemia intermedia encompasses a wide clinical spectrum of beta-thalassemia phenotypes. Some thalassemia intermedia patients are asymptomatic until adult life, whereas others are symptomatic from as young as 2 years of age. A number of clinical complications commonly associated with thalassemia intermedia are rarely seen in thalassemia major, including extramedullary hematopoiesis, leg ulce...

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Endocrine Function in Thalassemia Intermedia

UNLABELLED Thalassemias are the most common genetic disorder on a wordwide basis. β-thalassemia is a severe hemolytic anemia which results from genetic defects in the synthesis of the hemoglobin β-chain. Various endocrine abnormalities have been described in patients with thalassemia major. Endocrine disturbances have also been observed in patients with thalassemia intermedia (TI). In this stud...

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An update on thalassemia intermedia.

Thalassemia intermedia is a genetically diverse group of diseases that is the result of an imbalance in the production of the alpha and beta chains with ensuing chronic hemolysis, ineffective erythropoiesis, and iron overload. Resulting complications include bone changes, hypercoagulability, and end-organ damage due to iron overload. This decade has witnessed major breakthroughs in the manageme...

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Did the Proband Have Thalassemia Intermedia or Severe Thalassemia Trait?

I enjoyed reading Bilgen et al.’s case report, The effect of HBB: (c*+96T>C (3’UTR+1570 T>C) on the mild β-thalassemia intermedia pheonotype, in the recent issue of the Journal (2011; 28: 219-222). I congratulate the authors for exploring at the molecular level at least one of the thalassemia minima that fits well with the present clinical thalassemia nomenclature. Based on their clinical descr...

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ژورنال

عنوان ژورنال: Blood

سال: 1964

ISSN: 0006-4971,1528-0020

DOI: 10.1182/blood.v23.6.829.829