Hem/Onc Quiz #14: Congenital Anemias

1. A 6-month-old infant is brought to the pediatrician because of pallor and failure to thrive. The mother mentions that the baby seems very irritable and has had several episodes of fever without any apparent infection. Laboratory tests reveal anemia with an elevated reticulocyte count. A peripheral blood smear shows microspherocytes. Which of the following is the most likely diagnosis?

Question 1 of 5

2. 2-year-old boy is evaluated for persistent anemia. He was born to parents who are first cousins. Laboratory tests reveal a microcytic anemia with target cells noted on the peripheral smear. Genetic testing confirms a diagnosis affecting the synthesis of globin chains. Which of the following is the most likely diagnosis?

Question 2 of 5

3. A newborn is found to have severe anemia and is diagnosed with Diamond-Blackfan anemia. Which of the following is a characteristic finding associated with this condition?

Question 3 of 5

4. A 16-year-old girl with a known history of sickle cell anemia presents with fatigue, jaundice, and an acute vaso-occlusive crisis. Which of the following pathophysiologic mechanisms is primarily responsible for her hemolytic anemia?

Question 4 of 5

5. A 4-year-old boy with pallor and splenomegaly is diagnosed with congenital spherocytosis. Laboratory tests show increased mean corpuscular hemoglobin concentration (MCHC) and a negative direct Coombs test. What is the most effective definitive treatment for preventing further hemolytic episodes in this patient?

Question 5 of 5


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