Review Question - QID 109994

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Thalassemia Anemia Treatment β-thalassemia major QID: 109994 (M1.HE.17.4876)

QID 109994 (Type "109994" in App Search)

A 2-year-old boy is brought to the pediatrician by his mother because she is concerned that her son has not been growing as rapidly as her other children. They recently immigrated to the United States, and she is concerned that his diet in America has not been meeting his nutritional needs. Since immigrating, they have been living in an old house with several of their relatives. On presentation, his temperature is 98.7°F (37°C), blood pressure is 114/62 mmHg, pulse is 118/min, and respirations are 24/min. Based on clinical suspicion, a panel of labs are obtained as follows:

Hemoglobin: 10.2 g/dL
Hematocrit: 30.5%
Mean corpuscular volume: 71 um^3

A peripheral blood smear is also obtained and the results are shown in Figure A. Radiographs show an enlarged skull and prominent cheek bones. No radiodense lesions are seen in this patient. Which of the following treatments would most likely improve symptoms in this patient?

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Administration of succimer

11%

16/151

Frequent blood transfusions

56%

85/151

Increasing iron consumption

15%

23/151

Supplementation of vitamin B12

14/151

Treatment of underlying inflammation

3/151

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This child with a microcytic anemia, prominent cheekbones, and codocytes on blood smear most likely has beta-thalassemia major and should be treated with frequent blood transfusions.

Beta-thalassemia major is an autosomal recessive disorder found primarily in those of Mediterranean descent that causes a significant deficiency in hemoglobin beta chain production. 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. Specifically, patients with thalassemia have a microcytic anemia due to defective hemoglobin production and also have expansion of the skull and cheek bones in order to increase their capacity for extramedullary hematopoiesis. Frequent blood transfusions are needed for these children in order to alleviate their anemia.

Figure A shows a peripheral blood smear with microcytic, hypochromic target cells consistent with beta-thalassemia major.

Incorrect Answers:
Answer 1: Administration of succimer would be used in patients in lead poisoning anemia; however, these patients would have lead lines on radiograph and no expansion of extramedullary hematopoiesis.

Answer 3: Increasing iron consumption would be used in patients with iron deficiency anemia; however, these patients would not have target cells nor have expansion of extramedullary hematopoiesis.

Answer 4: Supplementation with vitamin B12 would be used in patients with B12 deficiency; however, these patients would present with a macrocytic anemia as well as neurologic defects.

Answer 5: Treatments of underlying inflammation would be used in patients with anemia of chronic disease; however, these patients would not have target cells nor have expansion of extramedullary hematopoiesis.

Bullet Summary:
Beta-thalassemia major causes a microcytic anemia with codocytes that should be treated with frequent blood transfusions.

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