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Splenectomy
6%
19/338
Folic acid supplementation
4%
12/338
Blood transfusion
20%
69/338
No treatment is necessary
54%
184/338
Iron supplementation
13%
44/338
Select Answer to see Preferred Response
The patient presents with asymptomatic microcytic anemia. Red blood cell studies and hemoglobin electrophoresis suggest beta-thalassemia minor, which does not require treatment. Beta-thalassemia affects the beta-globin chain, an important component of hemoglobin A. Beta-thalassemia major is homozygous for the beta chain defect and is characterized by severe anemia beginning several months after birth requiring blood transfusion. Beta-thalassemia minor is heterozygous for the defect and is often asymptomatic. Diagnosis of beta-thalassemia minor is usually an incidental finding of microcytic anemia. A mean corpuscular volume (MCV) of less than 75 fL and normal RDW can help distinguish the condition from iron deficiency anemia. Muncie and Campbell discuss the diagnosis, complications, and treatment of alpha and beta thalassemia. They suggest that the Mentzer index (MCV/red blood cell count) is useful to distinguish iron deficiency and thalassemia; the ratio is >13 in iron deficiency, and <13 in thalassemia. They also present an algorithm for the interpretation of RDW in the setting of microcytic anemia. Sheiner et al. analyzed the pregnancy outcomes in patients with thalassemia minor compared to controls. They concluded there were no differences in terms of birth weight, Apgar scores, congenital malformations, or perinatal mortality, although there was a higher rate of intrauterine growth restriction in mothers with thalassemia minor. Figure A shows microcytic, hypochromic red blood cells with many target cells. This is commonly seen in patients with beta thalassemia minor. Illustration A is an algorithm for diagnosing microcytic anemia. Incorrect answers: Answer 1: Splenectomy can be performed in beta-thalassemia major, but is not indicated in the minor form. Answer 2: Folic acid deficiency results in macrocytic anemia, whereas this patient has a normal RDW. Answer 3: Blood transfusion is required in beta thalassemia major, and usually results in iron overload syndromes over time. Answer 5: Iron supplementation is not indicated as the patient’s labs are not consistent with iron deficiency anemia becase RDW is normal (would be high with iron deficiency anemia).
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