Pathophysiology of Beta-Thalassaemia: (Part – 1) [Ineffective Erythropoiesis]

Thalassaemia is an inherited haemoglobin disorder characterized by reduced or absent globin chain synthesis, resulting in variable clinical phenotypes from severe chronic anaemia requiring lifelong transfusion and iron chelating therapy to asymptomatic indivi- duals. β-thalassaemia has a broad clinical spectrum, and traditionally has been classified as thalassaemia major (TM), thalassaemia intermedia (TI), and thalassaemia minor. Acc- ording to RBC/Blood transfusions requirement, β-thalassaemia is classified as either transfusion-dependent thalassaemia (TDT) or non-transfusion-dependent thalassaemia (NTDT). Patients with TDT need life-long regular transfusions for survival from early child- hood, while patients with NTDT do not need life-long regular transfusions for survival.