1. An otherwise healthy 50 y/o white female is undergoing a transthoracic echocardiogram to evaluate possible mitral regurgitation. To suppress the gag reflex lidocaine spray is used liberally on the posterior pharynx. As the procedure begins the patient is noted to become short of breath and anxious. Immediately the procedure is stopped and the physician notes the patient’s lips are blue. A blood gas is drawn, which shows a pO2 = 98 mmHg and a pCO2 = 30 mmHg. The best treatment of the patient would be?

2. Which of the following methods is most accurate in quantitating HbA2 (important in β-thalassemia)?

3. Given the findings in the peripheral blood smear in Case #4 in the images below, which of the following is the best diagnosis:

4. At what age is the best time to screen for sickle cell disorders?

5. The best diagnosis for the peripheral smear illustrated in Case #3 in the images below is:

6. The images for Case #1 below are characteristic of which of the following:

7. A 30 y/o Asian female is found to have a homozygous hemoglobinopathy. She has a hypochromic anemia and splenomegaly. Which of the following is the most likely the etiology?

8. A 70 y/o male presents with a WBC count of 2.1, Hb of 8.7 g/dL, and plts. of 88K. There is no evidence of hepatosplenomegaly or lymphadenopathy. Given this history and the image findings in the images below for Case #6 (blast percentage = 17%), which of the following is the most likely diagnosis?

9. When considering hemoglobin electrophoresis on cellulose acetate at pH 8.4, which of the following is the correct order of Hb migration from the cathode towards the anode?

10. Which of the following is the cause of target cells found in HbCC disease?

11. Which of the following is the best diagnosis given the findings in the peripheral smear in Case #2 below:

12. A 65 y/o male patient presents with increasing fatigue, a WBC count of 65K, and the findings illustrated on the peripheral blood smear in Case #5 in the images below. The most likely diagnosis is:

13. An amino acid substitution of lysine for glutamic acid in position 6 of the β hemoglobin molecule is characteristic of which Hb?