Question 1

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

Accepted Answer

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

Question 2

Why are blister cells NOT seen in PK deficiency?

Accepted Answer

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) All of the above 
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) All of the above A

Question 3

Which of the following is a quantitative test for G6PD?

Accepted Answer

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

Question 4

The majority of glucose catabolism occurs via the:

Accepted Answer

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

Question 5

Which molecule is responsible for the neutralization of harmful peroxides?

Accepted Answer

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

Question 6

What is the poikilocyte typically seen in PK deficiency?

Accepted Answer

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

Question 7

Which of the following is the most common RBC enzymopathy?

Accepted Answer

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

Question 8

Class IV G6PD enzymes include which of the following?

Accepted Answer

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

Question 9

The mode of inheritance for G6PD deficiency is:

Accepted Answer

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

Question 10

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

Accepted Answer

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

Question 11

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

Accepted Answer

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. 
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 12

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

Accepted Answer

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

Question 13

Intrinsic erythrocyte enzyme deficiencies lead to:

Accepted Answer

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

Question 14

The common erythrocyte enzyme disorder G6PD deficiency is due to a gene mutation on which chromosome?

Accepted Answer

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

Question 15

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

Accepted Answer

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

Question 16

Pyruvate kinase is responsible for the conversion of:

Accepted Answer

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

Question 17

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?

Accepted Answer

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

Question 18

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

Accepted Answer

Fluorescent spot test: Whole blood is ad

Question 19

Exposure to which of the following can induce hemolysis due to G6PD deficiency?

Accepted Answer

A) Primaquine 
B) Fava beans 
C) Divicine 
D) All of the above 
A) Primaquine 
B) Fava beans 
C) Divicine 
D) All of the above

Question 20

Bite cells are produced during :

Accepted Answer

A) Removal of Doehle 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. 
A) Removal of Doehle 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.

Exam 15: Hemolytic Anemia: Enzyme Deficiencies

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

Why are blister cells NOT seen in PK deficiency?

Which of the following is a quantitative test for G6PD?

The majority of glucose catabolism occurs via the:

Which molecule is responsible for the neutralization of harmful peroxides?

What is the poikilocyte typically seen in PK deficiency?

Which of the following is the most common RBC enzymopathy?

Class IV G6PD enzymes include which of the following?

The mode of inheritance for G6PD deficiency is:

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

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

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

Intrinsic erythrocyte enzyme deficiencies lead to:

The common erythrocyte enzyme disorder G6PD deficiency is due to a gene mutation on which chromosome?

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

Pyruvate kinase is responsible for the conversion of:

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?

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

Exposure to which of the following can induce hemolysis due to G6PD deficiency?

Bite cells are produced during :

Introduction

Cellular Homeostasis

Hematopoiesis

Structure and Function of Hematopoietic Organs

The Erythrocyte

Hemoglobin

The Leukocyte

Introduction to Anemia

Anemias of Disordered Iron Metabolism and Heme Synthesis

Hemoglobinopaties: Qualitative Defects

Thalassemia

Megaloblastic and Nonmegaloblastic Macrocytic Anemias

Introduction to Hemolytic Anemia

Hemolytic Anemia: Membrane Defects

Hemolytic Anemia: Immune Anemias

Hemolytic Anemia: Nonimmune Defects

Nonmalignant Granulocyte and Monocyte Disorders

Nonmalignant Lymphocyte Disorders

Introduction to Hematopoietic Neoplasms

Myeloproliferative Disorders

Myelodysplastic Syndromes

Acute Myeloid Leukemias

Acute Lymphoblastic Leukemias

Lymphoid Malignancies: Chronic Lymphoid Leukemias, Lymphomas, and Plasma Cell Neoplasms

Hematopoietic Stem Cell Transplantation

Morphologic Analysis of Body Fluids in the Hematology Laboratory

Primary Hemostasis

Secondary Hemostasis and Fibrinolysis

Disorders of Primary Hemostasis

Disorders of Secondary Hemostasis

Thrombophilia

Hematology Procedures and Hemostasis

Bone Marrow Examination

Automation in Hematology and Hemostasis

Flow Cytometry

Chromosome Analysis of Hematopoietic and Lymphoid Disorders

Molecular Analysis of Hematologic Diseases

Laboratory Testing in Coagulation

Quality Assessment in the Hematology Laboratory

Filters

Increased bilirubin in the blood can lead to , and is seen in _.