Question 1

What is a point mutation?

Accepted Answer

A) mutation of the stop codon 
B) replacement of one nucleotide in the normal gene with a different nucleotide 
C) addition of one nucleotide in the normal gene 
D) deletion of one nucleotide in the normal gene 
A) mutation of the stop codon 
B) replacement of one nucleotide in the normal gene with a different nucleotide 
C) addition of one nucleotide in the normal gene 
D) deletion of one nucleotide in the normal gene B

Question 2

Which of the following would be an unexpected finding for patients with Hb SC disease or S/B⁰⁻ thalassemia?

Accepted Answer

A) target cells 
B) shortened red cell survival 
C) normal tube solubility test 
D) mild to moderate clinical features 
A) target cells 
B) shortened red cell survival 
C) normal tube solubility test 
D) mild to moderate clinical features C

Question 3

Heinz bodies are found when hemoglobin

Accepted Answer

A) precipitates. 
B) has higher oxygen affinity. 
C) concentration is decreased. 
D) forms crystals. 
A) precipitates. 
B) has higher oxygen affinity. 
C) concentration is decreased. 
D) forms crystals. A

Question 4

The qualitative hemoglobinopathies cause disease by producing hemoglobin chains that are

Accepted Answer

A) too long. 
B) produced in inadequate amounts. 
C) structurally altered. 
D) missing. 
A) too long. 
B) produced in inadequate amounts. 
C) structurally altered. 
D) missing.

Question 5

All of the following improve the clinical outcome of sickle cell anemia (Hb SS)except which of the following?

Accepted Answer

A) aggressive prevention and treatment of infection 
B) presence of Hb F 
C) transfusions during crises 
D) keeping the oxygen saturation low 
A) aggressive prevention and treatment of infection 
B) presence of Hb F 
C) transfusions during crises 
D) keeping the oxygen saturation low

Question 6

Cellulose acetate is performed on a patient with known homozygous Hb S who has received red cell transfusions in the past week.What hemoglobins would be expected to be found?

Accepted Answer

A) Hb S and Hb F 
B) Hb A and Hb F 
C) Hb S, Hb A, and Hb F 
D) Hb S, Hb A, Hb F, and Hb A2 
A) Hb S and Hb F 
B) Hb A and Hb F 
C) Hb S, Hb A, and Hb F 
D) Hb S, Hb A, Hb F, and Hb A2

Question 7

A patient has 45% Hb S,55% Hb A,and a positive tube solubility.Which of the following would likely be found?

Accepted Answer

A) normal blood film and no disease 
B) target cells on the blood film and no disease 
C) normal blood film and severe disease 
D) sickle cells on the blood film and severe disease 
A) normal blood film and no disease 
B) target cells on the blood film and no disease 
C) normal blood film and severe disease 
D) sickle cells on the blood film and severe disease

Question 8

Select the best test to confirm the diagnosis of the patient in Question 9.

Accepted Answer

A) mean cell volume (MCV) 
B) osmotic fragility 
C) hemoglobin electrophoresis 
D) presence of sickle cells 
A) mean cell volume (MCV) 
B) osmotic fragility 
C) hemoglobin electrophoresis 
D) presence of sickle cells

Question 9

Which of the following would be an unusual finding in the blood film of an adult with sickle cell disease crisis?

Accepted Answer

A) Heinz bodies 
B) target cells 
C) Howell-Holly bodies 
D) leukocytosis 
A) Heinz bodies 
B) target cells 
C) Howell-Holly bodies 
D) leukocytosis

Question 10

Sickle cell disease is found in individuals descended from ancestors from all of the following places except

Accepted Answer

A) Africa 
B) Middle East 
C) the Mediterranean 
D) Scandinavia 
A) Africa 
B) Middle East 
C) the Mediterranean 
D) Scandinavia

Question 11

Which of the following would be unexpected in homozygous Hb S?

Accepted Answer

A) high reticulocyte count 
B) hemoglobin A (Hb 
A)  
C) elevated hemoglobin F (Hb F) 
D) hemoglobin that is less soluble 
A) high reticulocyte count 
B) hemoglobin A (Hb 
A)  
C) elevated hemoglobin F (Hb F) 
D) hemoglobin that is less soluble

Question 12

A patient has target cells and a positive tube solubility test.This patient probably

Accepted Answer

A) is normal. 
B) has sickle cell anemia. 
C) has sickle trait. 
D) has either sickle cell anemia or trait. 
A) is normal. 
B) has sickle cell anemia. 
C) has sickle trait. 
D) has either sickle cell anemia or trait.

Question 13

Sickle cell presence in the blood results in all of the following except

Accepted Answer

A) decrease in blood viscosity. 
B) decrease in oxygen tension. 
C) decrease in blood pH. 
D) increase in 2,3-biphosphoglycerate . 
A) decrease in blood viscosity. 
B) decrease in oxygen tension. 
C) decrease in blood pH. 
D) increase in 2,3-biphosphoglycerate .

Question 14

Which of the following is a typical finding in patients with Hb M?

Accepted Answer

A) severe anemia requiring repeated blood transfusions 
B) elevated levels of ferrous iron (Fe2+) in the red cells 
C) presence of Heinz bodies on Wright-stained blood film 
D) elevated methemoglobin 
A) severe anemia requiring repeated blood transfusions 
B) elevated levels of ferrous iron (Fe2+) in the red cells 
C) presence of Heinz bodies on Wright-stained blood film 
D) elevated methemoglobin

Question 15

What is the most likely causative association between malaria and sickle cell trait?

Accepted Answer

A) Sickle gene makes people more prone to malaria infection. 
B) Sickle gene prevents Plasmodium ovale infection. 
C) They are both found in Asia. 
D) Sickle gene offers some protection against malaria. 
A) Sickle gene makes people more prone to malaria infection. 
B) Sickle gene prevents Plasmodium ovale infection. 
C) They are both found in Asia. 
D) Sickle gene offers some protection against malaria.

Question 16

Moderate to marked target cells are found on a blood film.Which of the following can most likely be eliminated?

Accepted Answer

A) Hb C disease 
B) hereditary spherocytosis 
C) Hb E disease 
D) sickle cell disease 
A) Hb C disease 
B) hereditary spherocytosis 
C) Hb E disease 
D) sickle cell disease

Question 17

Which of the following hemoglobinopathies is most common?

Accepted Answer

A) sickle cell diseases 
B) Hb E disorders 
C) Hb C disorders 
D) unstable hemoglobins 
A) sickle cell diseases 
B) Hb E disorders 
C) Hb C disorders 
D) unstable hemoglobins

Question 18

An adult female patient from Southeast Asia has no anemia,but her complete blood count (CBC)shows an MCV of 68 fL and marked target cells are observed on her blood film.Cellulose acetate alkaline electrophoresis shows two large bands: approximately 55% migrating with Hb A and 40% migrating with Hb C.What is the patient's most likely genotype?

Accepted Answer

A) Hb A and Hb C 
B) Hb A and Hb E 
C) Hb C and Hb E 
D) homozygous Hb E 
A) Hb A and Hb C 
B) Hb A and Hb E 
C) Hb C and Hb E 
D) homozygous Hb E

Question 19

What is the correct relationship between red cells' affinity for oxygen and red cell production?

Accepted Answer

A) When affinity is low, more oxygen is released and the body compensates by making red cells larger. 
B) When affinity is low, less oxygen is released and the body compensates by increasing erythropoietin and making more red cells. 
C) When affinity is high, less oxygen is released and the body compensates by increasing erythropoietin and making more red cells. 
D) When affinity is high, more oxygen is released and fewer red cells are needed. 
A) When affinity is low, more oxygen is released and the body compensates by making red cells larger. 
B) When affinity is low, less oxygen is released and the body compensates by increasing erythropoietin and making more red cells. 
C) When affinity is high, less oxygen is released and the body compensates by increasing erythropoietin and making more red cells. 
D) When affinity is high, more oxygen is released and fewer red cells are needed.

Question 20

A patient has two bands on alkaline hemoglobin electrophoresis.One lines up with Hb A and the other is in the S position.Select the best course of action.

Accepted Answer

A) Report as normal. 
B) Report as sickle trait. 
C) Report as sickle disease. 
D) Confirm sickle gene with tube solubility test. 
A) Report as normal. 
B) Report as sickle trait. 
C) Report as sickle disease. 
D) Confirm sickle gene with tube solubility test.

Exam 27: Hemoglobinopathies (Structural Defects in Hemoglobin)

What is a point mutation?

Which of the following would be an unexpected finding for patients with Hb SC disease or S/B⁰⁻ thalassemia?

Heinz bodies are found when hemoglobin

The qualitative hemoglobinopathies cause disease by producing hemoglobin chains that are

All of the following improve the clinical outcome of sickle cell anemia (Hb SS)except which of the following?

Cellulose acetate is performed on a patient with known homozygous Hb S who has received red cell transfusions in the past week.What hemoglobins would be expected to be found?

A patient has 45% Hb S,55% Hb A,and a positive tube solubility.Which of the following would likely be found?

Select the best test to confirm the diagnosis of the patient in Question 9.

Which of the following would be an unusual finding in the blood film of an adult with sickle cell disease crisis?

Sickle cell disease is found in individuals descended from ancestors from all of the following places except

Which of the following would be unexpected in homozygous Hb S?

A patient has target cells and a positive tube solubility test.This patient probably

Sickle cell presence in the blood results in all of the following except

Which of the following is a typical finding in patients with Hb M?

What is the most likely causative association between malaria and sickle cell trait?

Moderate to marked target cells are found on a blood film.Which of the following can most likely be eliminated?

Which of the following hemoglobinopathies is most common?

What is the correct relationship between red cells' affinity for oxygen and red cell production?

A patient has two bands on alkaline hemoglobin electrophoresis.One lines up with Hb A and the other is in the S position.Select the best course of action.

An Overview of Clinical Laboratory Hematology

Safety in the Hematology Laboratory

Blood Specimen Collection

Care and Use of the Microscope

Quality Assurance in Hematology and Hemostasis Testing

Cellular Structure and Function

Hematopoiesis

Erythrocyte Production and Destruction

Erythrocyte Metabolism and Membrane Structure and Function

Hemoglobin Metabolism

Iron Kinetics and Laboratory Assessment

Leukocyte Development, Kinetics, and Functions

Platelet Production, Structure, and Function

Manual, Semiautomated and Point-of-Care Testing in Hematology

Automated Blood Cell Analysis

Examination of the Peripheral Blood Film and Correlation with the Complete Blood Count

Bone Marrow Examination

Body Fluid Analysis in the Hematology Laboratory

Anemias: Red Blood Cell Morphology and Approach to Diagnosis

Disorders of Iron Kinetics and Heme Metabolism

Anemias Caused by Defects of DNA Metabolism

Bone Marrow Failure

Introduction to Increased Destruction of Erythrocytes

Intrinsic Defects Leading to Increased Erythrocyte Destruction

Extrinsic Defects Leading to Increased Erythrocyte Destruction Nonimmune Causes

Extrinsic Defects Leading to Increased Erythrocyte Destruction Immune Causes

Thalassemias

Nonmalignant Leukocyte Disorders

Cytogenetics

Molecular Diagnostics in Hematopathology

Flow Cytometric Analysis in Hematologic Disorders

Myeloproliferative Neoplasms

Myelodysplastic Syndromes

Acute Leukemias

Mature Lymphoid Neoplasms

Normal Hemostasis and Coagulation

Hemorrhagic Disorders and Laboratory Assessment

Thrombotic Disorders and Laboratory Assessment

Thrombocytopenia and Thrombocytosis

Qualitative Disorders of Platelets and Vasculature

Laboratory Evaluation of Hemostasis

Antithrombotic Therapies and their Laboratory Assessment

Hemostasis and Coagulation Instrumentation

Pediatric and Geriatric Hematology and Hemostasis

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