Thalassemias are inherited anemias characterized by decreased or absent globin chain synthesis and by ineffective erythropoesis. They are classified according to the defective globin chain and to the clinical severity. Beta-thalassemias, which are prevalent in the Mediterranean region, have decreased or absent beta-globin chain synthesis whereas, in alpha-thalassemias, the alpha-chain is affected. Thalassemia major is a severe, transfusion-dependent anemia with significant morbidity and mortality. Thalassemia intermedia are a genetic variant of the beta-thalassemias, which incorporates a less severe anemia than thalassemia major with inefficient erythropoiesis as well as peripheral hemolysis. Leg ulcers, pulmonary arterial hypertension, extramedullary hematopoiesis, and thrombotic events are some of the complications that thalassemia intermedia patients face.
|Titolo:||Beta-thalassemia intermedia: an overview|
CAPPELLINI, MARIA DOMENICA (Secondo)
|Parole Chiave:||therapy; hydroxyurea; Iran|
|Settore Scientifico Disciplinare:||Settore MED/09 - Medicina Interna|
|Data di pubblicazione:||mag-2008|
|Appare nelle tipologie:||01 - Articolo su periodico|