Question 1

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

Accepted Answer

The purpose of the HMP shunt i

Question 2

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

Accepted Answer

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

Question 3

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

Accepted Answer

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

Question 4

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

Accepted Answer

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

Question 5

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

Accepted Answer

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

Question 6

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

Accepted Answer

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

Question 7

The erythrocyte morphology associated with pyruvate kinase deficiency is:

Accepted Answer

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

Question 8

Which G6PD variant has normal activity and no hemolysis present?

Accepted Answer

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

Question 9

Why are Heinz bodies seen in G6PD deficiency?

Accepted Answer

Oxidative crises associated wi

Question 10

Which of the following supports the diagnosis of G6PD deficiency?

Accepted Answer

A) Presence of Heinz bodies with new methylene blue stain 
B) Reticulocytosis 
C) Hemolytic morphology during oxidative event 
D) All of the above 
A) Presence of Heinz bodies with new methylene blue stain 
B) Reticulocytosis 
C) Hemolytic morphology during oxidative event 
D) All of the above

Question 11

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

Accepted Answer

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

Question 12

Why are echinocytes seen in PK deficiency?

Accepted Answer

PK deficiency impairs the production of

Question 13

How do Heinz bodies differ morphologically from other erythrocyte inclusions?

Accepted Answer

Heinz bodies typically attach

Question 14

G6PD deficiency was first recognized during the Korean War, when what drugs were administered to the soldiers?

Accepted Answer

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

Question 15

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

Accepted Answer

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

Exam 15: Hemolytic Anemia: Enzyme Deficiencies

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

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

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

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

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

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

The erythrocyte morphology associated with pyruvate kinase deficiency is:

Which G6PD variant has normal activity and no hemolysis present?

Why are Heinz bodies seen in G6PD deficiency?

Which of the following supports the diagnosis of G6PD deficiency?

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

Why are echinocytes seen in PK deficiency?

How do Heinz bodies differ morphologically from other erythrocyte inclusions?

G6PD deficiency was first recognized during the Korean War, when what drugs were administered to the soldiers?

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

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

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