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## Beta-Thalassemia - Have protrusion upper teeth and mongoloid facial features. - Physical growth and development delayed. ### Characteristics - Characterized by severe microcytic, hypochromic anemia (MCV in range of 50 to 60 fL). - PB shows markedly hypochromic, microcytic erythrocytes with ext...

## Beta-Thalassemia - Have protrusion upper teeth and mongoloid facial features. - Physical growth and development delayed. ### Characteristics - Characterized by severe microcytic, hypochromic anemia (MCV in range of 50 to 60 fL). - PB shows markedly hypochromic, microcytic erythrocytes with extreme poikilocytosis, such as target cells, teardrop cells and elliptocytes with basophilic stippling and numerous NRBCs. - High reticulocyte count (2-8%; low for the degree of anemia) - Most of hemoglobin present is Hb F with or without slight increase in HbA2. - Regular transfusions usually begin around one year of age and continue throughout life. ### Complications - Excessive number of transfusions results in iron overload and hemosiderosis; without iron chelation, patients develop cardiac disease. ### Treatment - Transfusion is the main line of treatment and carries the following hazards: - Development of iron overload. - Development of alloimmunization (antibodies to transfused RBCs). - Risk of transfusion-transmitted diseases - Bone marrow transplants may be future treatment, along with genetic engineering.