Question 1

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 D

Question 2

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 3

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 D

Question 4

What percentage of African Americans has heterozygous sickle cell trait?

Accepted Answer

A)  1% 
B)  8% 
C)  20% 
D)  40% 
A)  1% 
B)  8% 
C)  20% 
D)  40%

Question 5

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 6

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 7

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 8

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 9

What clinical feature of sickle cell disease accounts for the highest number of hospital admissions?

Accepted Answer

A)  Megaloblastic crisis 
B)  Vasoocclusion 
C)  Autosplenectomy 
D)  Hand-foot dactylitis 
A)  Megaloblastic crisis 
B)  Vasoocclusion 
C)  Autosplenectomy 
D)  Hand-foot dactylitis

Question 10

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 11

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 12

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

Question 13

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 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

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 16

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 17

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

Question 18

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 19

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

Question 20

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

Exam 26: Hemoglobinopathies Structural Defects in Hemoglobin

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

What is a point mutation?

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

What percentage of African Americans has heterozygous sickle cell trait?

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?

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

Which of the following hemoglobinopathies is most common?

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

What clinical feature of sickle cell disease accounts for the highest number of hospital admissions?

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

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

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

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

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

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.

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

Heinz bodies are found when hemoglobin:

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

An Overview of Clinical Laboratory Hematology

Safety in the Hematology Laboratory

Specimen Collection

Care and Use of the Microscope

Quality Assurance in Hematology and Hemostasis Testing

Cellular Structure and Function

Hematopoiesis

Erythrocyte Production and Destruction

Energy Metabolism and Membrane Physiology of the Erythrocyte

Hemoglobin Metabolism

Iron Metabolism

Leukocyte Development, Kinetics, and Functions

Platelet Production, Structure, and Function

Routine and Point-Of-Care Testing in Hematology: Manual and Semiautomated Methods

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

Bone Marrow Examination

Body Fluids in the Hematology Laboratory

Anemias: Red Blood Cell Morphology and Approach to Diagnosis

Disorders of Iron 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 Erythrocytedestructionnonimmune Causes

Extrinsic Defects Leading to Increased Erythrocytedestructionimmune Causes

Thalassemias

Nonmalignant Leukocyte Disorders

Introduction to Leukocyte Neoplasms

Cytochemistry

Cytogenetics

Molecular Diagnostics in the Clinical Laboratory

Flow Cytometric Analysis in Hematologic Disorders

Myeloproliferative Neoplasms

Myelodysplastic Syndromes

Acute Leukemias

Mature Lymphoid Neoplasms

Pediatric and Geriatric Hematology

Automated Cell-Counting Instrumentation

Normal Hemostasis and Coagulation

Hemorrhagic Coagulation Disorders

Thrombosis Risk Testing

Thrombocytopenia and Thrombocytosis

Qualitative Disorders of Platelets and Vasculature

Laboratory Evaluation of Hemostasis

Monitoring Antithrombotic Therapies

Coagulation Instrumentation

Filters

Which of the following would be an unexpected finding for patients with Hb SC disease or S/B^0-thalassemia?