Review Questions

1. What is the amino acid substitution in sickle cell anemia patients?

a. Adenine for thymine b. Lysine for valine c. Valine for glutamic acid d. Cytosine for guanine

2. Which of the following factors contributes to the pathophysiology of sickling?

a. Increased iron concentration b. Hypochromia c. Fava beans d. Dehydration

3. Which of the following statements pertain to most of the clinically significant hemoglobin variants?

a. Most are fusion hemoglobins.

b. Most are singe amino acid substitution.

c. Most are synthetic defects.

d. Most are extensions of the amino acid chain.

4. Which of the following hemoglobins ranks second in variant hemoglobins worldwide?

Hemoglobin S-beta thalassemia may produce conditions as severe as sickle cell disease.

Hemoglobin E is the third most prevalent hemoglobin variant and is seen with great frequency in the southeast Asian populations. Hemoglobin D and hemoglobin Gphila migrate with hemoglobin S on alkaline electrophphoilraesis.

5. Which hemoglobin will show crystals appearing like bars of gold in the peripheral smear?

a. Hemoglobin CC disease b. Hemoglobin DD disease c. Hemoglobin EE disease d. Hemoglobin SS disease

6. Which one of the following conditions is the leading cause of hospitalization for sickle cell patients?

a. Acute chest syndrome b. Priapism c. Painful crisis d. Splenic sequestration

7. Which of the following hemoglobin separation methods is used for most newborn hemoglobin screening?

a. High-performance liquid chromatography b. Alkaline electrophoresis c. Isoelectric focusing d. Acid electrophoresis from the time he was seen in the emergency department, he was admitted and put in the critical care unit, in grave condition. His breathing was compromised and he was placed on mechanical ventilation and lapsed into a coma. He developed disseminated intravascular coagulation (DIC), using 10 units of fresh frozen plasma, 10 units of platelets, and 20 units of packed cells to control the bleeding. Twenty-four hours after admission, he died from overwhelming sepsis. Initial results are as follows (see cover for normal values). What role does splenic function play in the management of sickle cell patients?

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