Deck 18: Hemolytic Anemia: Enzyme Deficiencies

Question 1

Why are blister cells not seen in PK deficiency?

A)Because Heinz bodies are not formed in PK deficiency
B)Because G6PD deficiency causes hemolysis,and PK does not
C)Because G6PD deficient erythrocytes are prone to oxidative damage,and PK deficient erythrocytes are not
D)Because PK deficiency results in the formation of acanthocytes not Heinz bodies

Answer 1

Because Heinz bodies are not formed in PK deficiency

Question 2

The presence of which poikilocyte in the peripheral blood could indicate G6PD deficiency?

A)Schistocyte
B)Spherocyte
C)Blister cell
D)Elliptocyte

Answer 2

Blister cell

Question 3

What metabolic pathway catabolizes approximately 10% of the glucose essential for maintaining adequate concentrates of reduced glutathione?

A)Glycolytic pathway
B)Metabolic pathway
C)Hexose-monophosphate shunt
D)Nucleotide pathway

Answer 3

Hexose-monophosphate shunt

Question 4

All of the following support the diagnosis of G6PD deficiency except:

A)Presence of Heinz bodies with new methylene blue stain
B)"Bite cells"
C)Hemolytic morphology during oxidative event
D)Reticulocytopenia

Question 5

What morphologic characteristics are found on a blood smear in G6PD deficiency?

A)Leukopenia and echinocytes
B)Thrombocytopenia and reticulocytosis
C)Reticulocytosis and blister cells
D)Döhle bodies and Heinz bodies

Question 6

Which of the following is a quantitative test for G6PD?

A)Fluorescent spot test
B)Quantitative NADP rate reduction test
C)NADP dye reduction test
D)Ascorbate cyanide test

Question 7

The majority of glucose catabolism occurs via the:

A)Hexose monophosphate shunt
B)Embden-Meyerhof pathway
C)Luebering-Rapoport pathway
D)Synthesis of nucleotides

Question 8

G6PD deficiency was first recognized during the Korean War when which of the following drugs were administered to the soldiers?

A)Sulfonamides
B)Nitrofurans
C)Analgesics
D)Antimalarials

Question 9

The common erythrocyte enzyme disorder G6PD deficiency results from a gene mutation on which chromosome?

A)23
B)9
C)X
D)Y

Question 10

Intrinsic erythrocyte enzyme deficiencies lead to:

A)Increased erythrocyte oxidative susceptibility
B)Exacerbated immune response against erythrocytes
C)Increased opsonization of erythrocytes
D)Increased extravascular hemolysis

Question 11

Which of the following is used to screen for G6PD deficiency?

A)Supravital stain
B)Fluorescent spot test
C)PB smear
D)Cytochemical staining

Question 12

Which of the following is the most common RBC enzymopathy?

A)PK
B)HK
C)G6PD
D)GPI

Question 13

What are the two main metabolic pathways that erythrocytes use to break down glucose?

A)Glycolytic and hexose monophosphate shunt
B)Glycolytic and Luebering-Rapoport pathway
C)Krebs cycle and electron transport system
D)Hexokinase and Krebs cycle

Question 14

Exposure to which of the following does not induce hemolysis from G6PD deficiency?

A)Primaquine
B)Fava beans
C)Divicine
D)Aspirin

Question 15

A 53-year-old Italian male was hospitalized with increased fatigue and hemoglobinuria.Patient history revealed his last ingested meal contained fava beans.G6PD levels were performed 3 months later,and the result was 5%.Based on these findings,which class of G6PD variant is most likely affected?

A)Class I
B)Class II
C)Class III
D)Class IV

Question 16

The mode of inheritance for G6PD deficiency is:

A)Autosomal recessive
B)Autosomal dominant
C)No pattern established
D)Sex linked

Question 17

Which molecule is responsible for the neutralization of harmful peroxides?

A)G6P
B)Glutathione
C)HK
D)PK

Question 18

Which of the following does not support the diagnosis of G6PD deficiency?

A)Presence of Heinz bodies with new methylene blue stain
B)Reticulocytosis
C)Hemolytic morphology during oxidative event
D)Decreased mean cell volume

Question 19

For proper diagnosis of G6PD deficiency,timing of the testing is critical.When should it occur?

A)Several months after a hemolytic attack
B)Within a week after a hemolytic attack
C)During compensatory reticulocytosis
D)After transfusion therapy

Question 20

Which of the following lab tests is used to confirm G6PD deficiency?

A)Fluorescent spot test
B)PB smear
C)NADP rate reduction test
D)Reticulocyte count

Question 21

What poikilocyte is typically seen in PK deficiency?

A)Heinz bodies
B)Acanthocytes
C)Spherocytes
D)Echinocytes

Question 22

In PK deficiency there is a loss of _______,which causes failure of the cation pumps in the RBC.

A)G6PD
B)PEP
C)Glucose
D)ATP

Question 23

How do Heinz bodies differ morphologically from other erythrocyte inclusions?

Question 24

The erythrocyte morphology associated with pyruvate kinase deficiency is:

A)Microcytic,hypochromic
B)Macrocytic,hypochromic
C)Normocytic,normochromic.
D)Normocytic,hypochromic

Question 25

What is the purpose of the HMP shunt,and why is it important?

Question 26

Bite cells are produced during:

A)Removal of Döhle bodies by macrophages
B)Passage of RBCs through fibrin strands
C)Removal of Holly-Jolly bodies by the spleen
D)Removal of Heinz bodies by macrophages

Question 27

Increased bilirubin in the blood of infants can lead to ________,and is seen in _________.

A)Hemoglobinuria;acute,acquired hemolytic anemia
B)Hemosiderinuria;congenital,nonspherocytic hemolytic anemia
C)Kernicterus;neonatal hyperbilirubinemia
D)Kernicterus;congenital,nonspherocytic hemolytic anemia

Question 28

Why are echinocytes seen in PK deficiency?

Question 29

Pyruvate kinase is responsible for the conversion of:

A)NADPH to ATP
B)Glucose to glucose 6 phosphate
C)PEP (phosphoenol pyruvate)to pyruvic acid
D)Pyruvic acid to lactic acid

Question 30

Which G6PD variant has normal activity and no hemolysis present?

A)G6PD-Mediterranean
B)Minnesota
C)Iowa
D)G6PD-B

Question 31

Why are Heinz bodies seen in G6PD deficiency?

Question 32

Female heterozygotes for G6PD deficiency have one population of cells that is normal and one that is G6PD deficient because:

A)There is random inactivation of one X chromosome
B)There is random inactivation of one Y chromosome.
C)There is random activation of one X chromosome.
D)There is random activation of one Y chromosome.

Question 33

Class IV G6PD enzymes include which of the following?

A)G6PD-Mediterranean
B)G6PD-B and G6PD-A+
C)G6PD-Iowa
D)G6PD-Canton

Question 34

PK deficiency causes which of the following to occur in the erythrocyte?

A)Denatured hemoglobin to precipitate
B)Splenic sequestration of normal erythrocytes
C)Premature apoptosis from lack of ATP formed
D)Membrane instability with excessive sodium and calcium influx

Question 35

Which of the following can result from a deficiency of pyruvate kinase in the erythrocyte?

A)Gain of ATP
B)Erythrocyte membrane failure with loss of potassium
C)Sodium and calcium depletion
D)Overhydration

Question 36

What are the principles of the following tests?
a.Fluorescent spot test
b.Dye reduction test
c.Ascorbate cyanide test
d.Rate reduction test