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Which of the following is characteristic of the peripheral blood smear in hemoglobin SC disease?


A) Target cells
B) Folded red cells
C) Occasional glove-shaped intracellular
D) Normocytic normochromic anemia
E) All of the above

F) B) and D)
G) A) and D)

Correct Answer

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Which of the following may be found on the hemoglobin electrophoresis at alkaline pH?


A) 95% HbC plus HbA2
B) No HbA (0%)
C) Less than 7% HbF
D) All of the above
E) None of the above

F) All of the above
G) A) and E)

Correct Answer

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Which of the following is characteristic of the hemoglobin electrophoresis at alkaline pH for hemoglobin SC disease?


A) 50% HbS
B) 50% HbC
C) <2% HbF
D) All of the above
E) None of the above

F) C) and D)
G) B) and D)

Correct Answer

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Which of the following is characteristic of hemoglobin D disease?


A) Most common variant in American blacks is HbD Punjab (HbD Los Angeles)
B) Less than 0.02% frequency in American blacks
C) Both homozygous and heterozygous states are asymptomatic
D) Peripheral blood smear is unremarkable, except for a few target cells
E) All of the above

F) B) and E)
G) C) and E)

Correct Answer

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Which of the following amino acid substitution occurs in hemoglobin S?


A) Valine for glutamic acid
B) Glutamic acid for valine
C) Lysine for glutamic acid
D) Glutamic acid for lysine
E) None of the above

F) A) and E)
G) B) and D)

Correct Answer

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Which of the following describes dactylitis, which commonly occurs in infants and young children with sickle cell anemia?


A) Microinfarction of small bones
B) Unequal bone growth
C) Painful swelling of the hands and feet
D) Any of the above
E) None of the above

F) All of the above
G) A) and D)

Correct Answer

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Which of the following represents globin chains that may combine with alpha chains to form a particular hemoglobin?


A) Beta ()
B) Gamma ()
C) Delta ()
D) Epsilon ()
E) All of the following

F) A) and B)
G) None of the above

Correct Answer

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Which of the following cannot be used as a screening test for hemoglobin S in newborns?


A) Tube solubility tests
B) Sodium metabisulfite sickle prep
C) Hemoglobin electrophoresis
D) A and C
E) A and B

F) A) and B)
G) A) and E)

Correct Answer

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Which of the following is not characteristic of hemoglobin D disease?


A) Migrates electrophoretically in same position as hemoglobin S
B) Less than 0.02% frequency in American blacks
C) Both homozygous and heterozygous states are asymptomatic
D) Peripheral blood smear is unremarkable, except for a few target cells
E) Positive tube solubility test

F) A) and B)
G) A) and C)

Correct Answer

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Which of the following hematological results will suggest an individual with sickle cell trait?


A) Peripheral blood smear is normal with the exception of target cells
B) Solubility screening tests are positive
C) Hemoglobin electrophoresis demonstrates hemoglobin A, hemoglobin S, and usually slightly elevated hemoglobin A2
D) Sickle cells present on the peripheral smear
E) A, B, and C

F) A) and B)
G) B) and E)

Correct Answer

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Hemoglobin C disease is found almost exclusively in which population?


A) Caucasian
B) Black
C) Asian
D) Oriental
E) All of the above

F) A) and B)
G) C) and D)

Correct Answer

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Which of the following can precipitate or promote sickling of red blood cells?


A) Decrease in oxygenation
B) Decrease in pH
C) Dehydration
D) All of the above
E) None of the above

F) A) and E)
G) C) and D)

Correct Answer

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Which of the following represents embryonic hemoglobins formed only in the embryonic stages of human development?


A) (22)
B) (22)
C) (22)
D) All of the above
E) None of the above

F) C) and D)
G) A) and B)

Correct Answer

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Which of the following may be found on the hemoglobin electrophoretic pattern for sickle cell anemia?


A) No HbA (0%)
B) 80-100% HbS
C) 1-20% HbF
D) 2-4% HbA2
E) All of the above

F) A) and D)
G) B) and E)

Correct Answer

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Which of the following remains the major cause of morbidity and mortality in patients with sickle cell anemia?


A) Serious bacterial infections
B) Renal papillary necrosis
C) Bone marrow aplasia
D) Infarctions
E) Cardiomegaly

F) B) and D)
G) A) and B)

Correct Answer

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What hemoglobin(s) is/are present in sickle cell trait?


A) HbA2
B) HbS
C) HbA
D) A and B
E) B and C

F) None of the above
G) A) and B)

Correct Answer

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The most significant cause of death during early childhood is the severe overwhelming septicemia and meningitis due to what organism?


A) Rubeola
B) Streptococcus pneumoniae
C) Cytomegalovirus
D) Histoplasma capsulatum
E) Staphylococcus aureus

F) A) and E)
G) A) and C)

Correct Answer

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Which abnormal hemoglobin migrates in the same position as HbA2 on hemoglobin electrophoresis at alkaline pH?


A) HbF
B) HbC
C) HbS
D) HbG
E) HbD

F) None of the above
G) All of the above

Correct Answer

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Which of the following is characteristic of the vaso-occlusive or painful crisis in a patient with sickle cell anemia?


A) Severe pain
B) Tissue damage and necrosis
C) Regional hypoxia and acidosis
D) Dehydration or exposure to extreme cold
E) All of the above

F) D) and E)
G) B) and E)

Correct Answer

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Which of the following represent(s) inherited high-affinity hemoglobins?


A) Hb Chesapeake
B) Hb Rainier
C) Hb Bethesda
D) Hb Hiroshima
E) All of the above

F) D) and E)
G) A) and B)

Correct Answer

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