American Board of Internal Medicine (ABIM) Certification Practice Exam

What is the initial treatment for β-thalassemia with hemoglobin levels less than 7 g/dL?

1. Bone marrow transplantation

2. Monthly erythrocyte transfusion

3. Oral iron supplements

4. Intravenous iron therapy

The correct answer is: Monthly erythrocyte transfusion

The initial treatment for β-thalassemia in patients with hemoglobin levels less than 7 g/dL typically involves monthly erythrocyte transfusions. This approach aims to manage severe anemia and prevent complications related to inadequate oxygen delivery to tissues. Erythrocyte transfusions provide a rapid increase in hemoglobin levels and improve the patient's overall well-being. In β-thalassemia, the body has a reduced ability to produce hemoglobin due to a genetic defect, leading to ineffective erythropoiesis and resultant anemia. Therefore, providing supplemental erythrocytes is crucial for maintaining sufficient oxygen-carrying capacity, particularly in individuals presenting with significant anemia. While bone marrow transplantation can be a definitive treatment option, it is generally considered for more severe cases or in younger patients with matched donors, rather than as an immediate initial treatment for low hemoglobin levels. Oral iron supplements and intravenous iron therapy are contraindicated in this scenario because patients with β-thalassemia often develop secondary iron overload from repeated transfusions, making iron supplementation potentially harmful. Instead, iron chelation therapies may be necessary to manage iron overload in patients who are receiving regular transfusions. Thus, monthly erythrocyte transfusion stands out as the most appropriate