Thelansis

  Beta-thalassemia (BT) is a genetic disorder characterized by a deficiency (Beta+) or absence (Beta0) of the beta-globin chains of hemoglobin (Hb). There are three main types of BT: BT-minor, BT-major, and BT-intermedia. BT-minor is the heterozygous form and is generally asymptomatic. At the same time, BT-major is the homozygous form that causes splenomegaly, microcytic, and hypochromic anemia and requires systematic transfusions to maintain Hb levels above 90-100 g/L. Long-term transfusion results in iron overload, which can cause significant morbidity and hamper the vital prognosis. BT-intermedia is less severe than BT-major and diagnosed later in life, and patients may or may not require occasional transfusions. Diagnosis of BT relies on analyzing Hb by electrophoresis or HPLC. Treatment options for BT include a combination of regular transfusions and iron chelation therapy, which has led to increased survival during the last 40 years. The prognosis depends on the severity of...

 Beta-thalassemia (BT) is a genetic disorder characterized by a deficiency (Beta+) or absence (Beta0) of the beta-globin chains of hemoglobin (Hb). There are three main types of BT: BT-minor, BT-major, and BT-intermedia. BT-minor is the heterozygous form and is generally asymptomatic. At the same time, BT-major is the homozygous form that causes splenomegaly, microcytic and hypochromic anemia, and requires systematic transfusions to maintain Hb levels above 90-100 g/L. Long-term transfusion results in iron overload, which can cause significant morbidity and hamper the vital prognosis. BT-intermedia is less severe than BT-major and diagnosed later in life, and patients may or may not require occasional transfusions. Diagnosis of BT relies on analyzing Hb by electrophoresis or HPLC. Treatment options for BT include a combination of regular transfusions and iron chelation therapy, which has led to increased survival during the last 40 years. The prognosis depends on the severity of th...