Thelansis

 β-thalassemia is typically a result of a monogenic disorder, primarily caused by a decrease or absence in the synthesis of the β-globin chain. This particular chain is vital to adult hemoglobin (HbA, α2β2). Consequently, an imbalance occurs between α and β chains, accumulating unattached α-globin chains. These excess α-globin chains form insoluble Hemi-chromes within erythrocyte precursor cells, causing damage to the erythrocyte membrane. This damage, in turn, triggers severe intramedullary erythrocyte apoptosis, referred to as ineffective erythropoiesis (IE). As a result, red blood cells (RBCs) experience a significantly shortened lifespan due to extra-medullary hemolysis, leading to severe anemia. The most severe manifestation of this condition, known as transfusion-dependent β-thalassemia (TDT), is characterized by profound anemia. To maintain adequate hemoglobin levels, individuals with TDT require lifelong and regular blood transfusions. However, these transfusions pose a con...

 β-thalassemia are monogenic disorders usually caused by reduced or absent synthesis of the βglobin chain, one of the main components of adult hemoglobin (HbA, α2β2. This results in a relative increase in the unattached αglobin chains (α/β-chain imbalance) that form insoluble Hemi-chromes in the erythrocyte progenitors. The Hemi-chromes damage the erythrocyte membrane, leading to severe intramedullary erythrocyte apoptosis (ineffective erythropoiesis, IE) and severely shortened red blood cell (RBC) life span due to extra-medullary hemolysis, leading to severe anemia. ·        Transfusion-dependent β-thalassemia (TDT) is the most severe form of the disease, characterized by severe anemia, and requires lifelong, regular blood transfusions to maintain Hb levels. These transfusions carry the risk of progressive multi-organ damage due to unavoidable iron overload. ·        In the USA, the estimated β-thalassemia cases range...

  β-thalassemia are monogenic disorders usually caused by reduced or absent synthesis of the βglobin chain, one of the main components of adult hemoglobin (HbA, α2β2. This results in a relative increase in the unattached αglobin chains (α/β-chain imbalance) that form insoluble Hemi-chromes in the erythrocyte progenitors. The Hemi-chromes damage the erythrocyte membrane, leading to severe intramedullary erythrocyte apoptosis (ineffective erythropoiesis, IE) and severely shortened red blood cell (RBC) life span due to extra-medullary hemolysis, leading to severe anemia. ·        Transfusion-dependent β-thalassemia (TDT) is the most severe form of the disease, characterized by severe anemia, and requires lifelong, regular blood transfusions to maintain Hb levels. These transfusions carry the risk of progressive multi-organ damage due to unavoidable iron overload ·        In the USA the estimated β-thalassemia cases range ...