The role of ineffective erythropoiesis in non-transfusion-dependent thalassemia
نویسندگان
چکیده
منابع مشابه
The role of ineffective erythropoiesis in non-transfusion-dependent thalassemia.
Ineffective erythropoiesis is the hallmark of beta-thalassemia that triggers a cascade of compensatory mechanisms resulting in clinical sequelae such as erythroid marrow expansion, extramedullary hematopoiesis, splenomegaly, and increased gastrointestinal iron absorption. Recent studies have begun to shed light on the complex molecular mechanisms underlying ineffective erythropoiesis and the as...
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In humans, β -thalassemia dyserythropoiesis is characterized by expansion of early erythroid precursors and erythroid progenitors and then ineffective erythropoiesis. This ineffective erythropoiesis is defined as a suboptimal production of mature erythrocytes originating from a proliferating pool of immature erythroblasts. It is characterized by (1) accelerated erythroid differentiation, (2) ma...
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Background: The development of anti-red blood cell alloantibodies remains a major problem in transfusion of blood in thalassemia major patients. Also, Autoantibodies can result in clinical hemolysis and difficulty in cross-matching blood. We studied the frequency of red blood cell alloimmunization and autoimmunization among thalassemia patients who received regular transfusions in Ilam province...
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beta-thalassemia encompasses a group of monogenic diseases that have in common defective synthesis of beta-globin. The defects involved are extremely heterogeneous and give rise to a large phenotypic spectrum, with patients that are almost asymptomatic to cases in which regular blood transfusions are required to sustain life. As a result of the inefficient synthesis of beta-globin, the patients...
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ژورنال
عنوان ژورنال: Blood Reviews
سال: 2012
ISSN: 0268-960X
DOI: 10.1016/s0268-960x(12)70005-x