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Frequent blood transfusions
50%
1/2
Iron chelation therapy
Bone marrow transplant
0%
0/2
Splenectomy
Growth hormone therapy
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This child has beta-thalassemia. Growth hormone therapy has had only variable success in these patients and is not typically used in their management. Beta-thalassemia major is an autosomal recessive disorder, more common in those with Mediterranean descent, that causes a significant deficiency in Hb A and beta chain production, resulting in severe hemolysis and ineffective erythropoiesis. Babies with this disorder appear normal at birth because of the prevalence of fetal hemoglobin (two alpha chains and two gamma chains); however, they may present with poor growth, anemia, and skeletal abnormalities starting around 6 months of age. As a result of frequent hemolysis and serial blood transfusions, iron levels increase and sometimes require chelation. Patients with beta-thalassemia might require splenectomy secondary to frequent transfusion requirements putting them at great risk of infection with encapsulated bacteria. Muncie and Campbell discuss the diagnosis of thalassemia versus iron deficiency-related microcytic anemia using the mean corpuscular volume (MCV), red blood cell distribution width (RDW). In thalassemia, the MCV is usually <75 fL. In iron deficiency anemia, the MCV is rarely <80 fL until the hematocrit is less than 30 percent. They report that in children, the Mentzer index (MCV/red blood cell count) can help distinguish between iron deficiency (>13) and thalassemia (<13). Schrier and Angelucci describe the various treatments of beta-thalassemia and those on the horizon. They report that the most anemic patients (intermedia and major) require regular red blood cell transfusions to prevent cardiac failure. They lament, however, that the inevitable iron accumulation leads to dysfunction involving the heart, liver, and endocrine system; thus, necessitating regular iron chelation. Allogeneic bone marrow (or stem cell) transplantation in severely affected subjects with both alpha and beta thalassemia has been suggested as a cure with targeted gene therapy close behind. Figure A demonstrates the microcytic, hypochromic target cells on blood smear indicative of thalassemia. Incorrect Answers: Answers 1-4 are all treatment modalities that have been shown to be effective in the management of beta-thalassemia.
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