10. Hemolytic Anemias: Intracorpuscular Defects: II. Hereditary Enzyme Deficiencies

Question 1

1.What is the most common glycolytic enzyme deficiency associated with the pentose phosphate pathway (aerobic pathway)?
a.PK deficiency
b.G6PD deficiency
c.Hexokinase deficiency
d.Glutathione reductase deficiency

Answer 1

b.G6PD deficiency

Question 2

2.What is the most common glycolytic enzyme deficiency associated with the Embden-Meyerhof pathway (anaero-bic pathway)?

a.PK deficiency
b.G6PD deficiency
c.Hexokinase deficiency
d.Glutathione reductase deficiency

Answer 2

a.PK deficiency

Question 3

3. Oxidative denaturation of hemoglobin results in formation of small particles that are visualized with supravital stain-ing. What is the term for these particles?

a. Basophilic stippling
b.Howell-Jolly bodies
c. Pappenheimer bodies
d.Heinz bodies

Answer 3

d.Heinz bodies

Question 4

4. In the evaluation of a patient for G6PD deficiency,which of the following test results would indicate a deficiency of the enzyme?

a. Increased formation of Heinz bodies
b. Lack of fluorescence in the fluorescnt spot test
c. Failure to reduce methemoglobin in the presence of methylene blue
d.All of the above

Answer 4

d.All of the above

Question 5

5. Which laboratory test result would indicate a patient who is PK-deficient?

a.Abnormal rate of hemolysis that is independent of the presence or absence of glucose in the incubation medium of the autohemolysis test
b. Lack of fluorescence in the fluorescent spot test
c. A change in the indicator from red to yellow in the orthocresol red test
d. Increase in osmotic fragility

Answer 5

a.Abnormal rate of hemolysis that is independent of the presence or absence of glucose in the incubation medium of the autohemolysis test

Question 6

6. What deficiency causes hemoglobin to be oxidized from the ferrous to the ferric state?

a.G6PD deficiency
b.PK deficiency
c. NADH-methemoglobin reductase deficiency
d. Lactate dehydrogenase deficiency

Answer 6

c. NADH-methemoglobin reductase deficiency