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Heart disease is the leading cause of mortality and one of the main causes of morbidity in beta-thalassemia. The clinical spectrum of the thalassemia syndrome ranges from the severe, transfusion--dependent thalassemia major and the asymptomatic carrier state. Thalassemia intermedia represents a milder form and is usually transfusion-independent. Two main(More)
BACKGROUND beta-Thalassemia is an inherited hemoglobin disorder characterized by reduced synthesis of beta-globin chain. The severity of clinical course distinguishes this heterogeneous disease in two main subtypes: thalassemia major (TM) and thalassemia intermedia (TI). TI has a later clinical onset with a milder anemia that does not require transfusions(More)
BACKGROUND Thalassemia intermedia has a later clinical onset and a milder anemia than thalassemia major, characterized by high output state, left ventricle remodeling, and age-related pulmonary hypertension. Bone deformities, extramedullary hematopoiesis (EMH), and spleen and liver enlargement are the consequences of hypoxia and enhanced erythropoiesis. The(More)
The homozygous beta-thalassemias are a group of genetically inherited hemoglobin (Hb) disorders characterized by dyserythropoietic anemia. According to the degree of anemia, two main forms, sharing a common basic molecular mechanism, are distinguished: thalassemia major (TM) and thalassemia intermedia (TI). The severity of the clinical phenotype(More)
Combination chelation therapy with desferrioxamine and deferiprone has recently been suggested as a more effective tissue iron-chelating treatment for transfusion-dependent beta-thalassemia patients, although a standard dosage protocol has not yet been established. We describe a thalassemia major patient who had been treated with combination therapy with(More)
An acquired diffuse elastic tissue defect that resembles inherited pseudoxanthoma elasticum (PXE) has been noticed with a significant age-related frequency in hemoglobin disorders, especially beta-thalassemia and has been held responsible for a number of complications observed in these cases, some of which are quite severe. We report here two patients with(More)
Dear Editor Intervertebral disc calcification (IDC) is a rare age-related phenomenon of variable clinical significance [3, 12], and hemochromatosis has been often associated with IDC, due to iron overload toxicity [1, 3, 7, 12–14]. Although hemolytic anemias are characterized by excessive tissue iron deposition and secondary hemosiderosis, only one case of(More)
Thalassemia intermedia is a heterogeneous, transfusion-independent form of b-thalassemia, with a clinical course dominated by multi-organ effects of chronic tissue hypoxia, in which hemoglobin F percentage seems to play an important role. We describe the case of a transfusion-independent thalassemia intermedia patient (total hemoglobin 10.7 g/dl) with high(More)
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