sandra
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18. A 10-month-old boy with hemoglobin SS disease is
brought to the emergency department by his parents
because of severe pain in his feet for 2 hours. He
appears acutely ill and is crying. Vital signs are
normal except for a pulse of 160/min. Physical
examination shows splenomegaly and edematous, tender
feet. Laboratory studies show a hemoglobin
concentration of 7 g/dL and a reticulocyte count of
12%. A peripheral blood smear shows target cells,
poikilocytes, and sickled erythrocytes. Which of the
following processes most likely caused the sickled
erythrocytes in this patient?
A. Decreased interaction of hemoglobin S with spectrin
upon deoxygenation
B. Inability of abnormal beta-chains to form tetramers
upon deoxygenation
C. Inability of abnormal beta-chains to form tetramers
upon oxygenation
D. Increased interaction of hemoglobin S with spectrin
upon oxygenation
E. Spontaneous polymerization of hemoglobin S upon
deoxygenation
F. Spontaneous polymerization of hemoglobin S upon
oxygenation
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