A 3-year-old boy diagnosed with thalassemia major receives multiple transfusions to maintain his blood hemoglobin level. On physical examination, his liver and spleen are mildly enlarged. He also has glucose intolerance as demonstrated by an oral glucose tolerance test. Which of the following therapies would prevent congestive cardiac failure in this patient?

Question

A 3-year-old boy diagnosed with thalassemia major receives multiple transfusions to maintain his blood hemoglobin level.  On physical examination, his liver and spleen are mildly enlarged.  He also has glucose intolerance as demonstrated by an oral glucose tolerance test.  Which of the following therapies would prevent congestive cardiac failure in this patient?

Quizplus · Accepted Answer

Many hemolytic diseases (eg, thalassemia major) require frequent, regular treatment with red blood cell transfusions for the maintenance of an adequate hemoglobin level. Over time, these chronic red blood cell transfusions cause iron to accumulate within the reticuloendothelial system and organs such as the liver and heart. Because iron cardiotoxicity may result in arrhythmias and congestive heart failure, it is important to implement chelation therapy within one to two years of beginning red blood cell transfusions. Chelation therapy allows for the removal of excessive iron from the body, and can delay or prevent the development of cardiac disease. Deferoxamine is one of the more effective and safe iron chelators, as it complexes with ferric ions to form ferrioxamine, which is removed by the kidneys. (Choices A, B, D, and E) Erythropoietin induces erythropoiesis and reticulocyte release from the bone marrow. It is commonly used to treat the anemia of chronic kidney disease. Cobalamin (vitamin B₁₂) is necessary in the treatment of pernicious anemia. Ascorbic acid (vitamin C) is used in the treatment of scurvy. Digoxin causes increased cardiac contractility, enhanced vagal tone, and decreased ventricular rate. As such, it is used in the treatment of congestive heart failure and supraventricular arrhythmias. None of these therapies are capable of preventing congestive heart failure secondary to iron cardiotoxicity, however. Educational objective: Chelation therapy with deferoxamine should be implemented in patients receiving chronic red blood cell transfusions. Deferoxamine prevents iron-induced cardiotoxicity and congestive heart failure from developing in this patient population.

A 3-Year-Old Boy Diagnosed with Thalassemia Major Receives Multiple Transfusions