Question 1

Both hemoglobin D and G migrate with which hemoglobin on alkaline gel electrophoresis?

Accepted Answer

A)  S 
B)  A 
C)  F 
D)  C 
A)  S 
B)  A 
C)  F 
D)  C

Question 2

Patients with sickle cell disease and those with sickle cell trait differ greatly in clinical presentation. List at least two features in clinical presentation, and explain why they differ.

Accepted Answer

Patients with sickle cell disease report

Question 3

Electrophoresis at an acid pH using a citrate agar gel separates what hemoglobin variants that travel together on cellulose acetate at pH 8.6?

Accepted Answer

A)  Hemoglobins A and S 
B)  Hemoglobins S and C 
C)  Hemoglobins D and S 
D)  Hemoglobins E and A 
A)  Hemoglobins A and S 
B)  Hemoglobins S and C 
C)  Hemoglobins D and S 
D)  Hemoglobins E and A

Question 4

Patients with sickle cell disease suffer from vaso-occlusive crises because:

Accepted Answer

A)  The altered hemoglobin facilitates the formation of bar-shaped hemoglobin crystals that occlude blood vessels. 
B)  The altered hemoglobin impairs the hemoglobin's ability to effectively transport oxygen that causes vaso-occlusion. 
C)  The altered hemoglobin facilitates tactoid crystal formation that alters the shape of the red blood cell, resulting in impeded blood flow in vessels. 
D)  The altered hemoglobin interferes with globin chain assembly, which impedes blood flow in the vessels. 
A)  The altered hemoglobin facilitates the formation of bar-shaped hemoglobin crystals that occlude blood vessels. 
B)  The altered hemoglobin impairs the hemoglobin's ability to effectively transport oxygen that causes vaso-occlusion. 
C)  The altered hemoglobin facilitates tactoid crystal formation that alters the shape of the red blood cell, resulting in impeded blood flow in vessels. 
D)  The altered hemoglobin interferes with globin chain assembly, which impedes blood flow in the vessels.

Question 5

Which of the following has the highest prevalence worldwide?

Accepted Answer

A)  Hgb S 
B)  Hgb C 
C)  Hgb D 
D)  Hgb E 
A)  Hgb S 
B)  Hgb C 
C)  Hgb D 
D)  Hgb E

Question 6

Sickle cell trait is not as severe as sickle cell anemia, so why is the sickle cell trait important to diagnose?

Accepted Answer

A)  One in four children born to trait parents have the disease. 
B)  Sickle cell trait can mask other diseases. 
C)  Doing so eliminates the need for pharmacologic agent treatment. 
D)  Cells still sickle under the same conditions as with the disease. 
A)  One in four children born to trait parents have the disease. 
B)  Sickle cell trait can mask other diseases. 
C)  Doing so eliminates the need for pharmacologic agent treatment. 
D)  Cells still sickle under the same conditions as with the disease.

Question 7

If a patient had hemoglobin S and hemoglobin A identified by electrophoresis and a positive hemoglobin solubility test, what is the patient's most likely condition?

Accepted Answer

A)  Hemoglobin C trait 
B)  Hemoglobin S/D disease 
C)  Hemoglobin SC disease 
D)  Sickle cell trait 
A)  Hemoglobin C trait 
B)  Hemoglobin S/D disease 
C)  Hemoglobin SC disease 
D)  Sickle cell trait

Question 8

Explain how methemoglobin is formed and how its presence impacts oxygen delivery. Be sure to include oxygen affinity in your answer.

Accepted Answer

Methemoglobin is formed by oxidation of

Question 9

Which laboratory method detects hemoglobinopathies?

Accepted Answer

A)  Spectrophotometric analysis at 450 nm 
B)  Careful examination of peripheral blood cellular components 
C)  Separation and quantification of protein subunits through an electrical field 
D)  Turbidimetric analysis of whole blood 
A)  Spectrophotometric analysis at 450 nm 
B)  Careful examination of peripheral blood cellular components 
C)  Separation and quantification of protein subunits through an electrical field 
D)  Turbidimetric analysis of whole blood

Question 10

Clinical disease that results from a genetic mutation in one or more genes that affects hemoglobin synthesis is termed:

Accepted Answer

A)  Thalassemia 
B)  Hemoglobinopathy 
C)  Porphyria 
D)  Asynchrony 
A)  Thalassemia 
B)  Hemoglobinopathy 
C)  Porphyria 
D)  Asynchrony

Question 11

The defect that will produce hemoglobin C disease is:

Accepted Answer

A)  Beta 6 substitution of glutamic acid for valine 
B)  Beta 6 substitution of lysine for glutamic acid 
C)  Alpha 26 substitution of glutamic acid for threonine 
D)  Alpha 4 substitution of thymidine for methionine 
A)  Beta 6 substitution of glutamic acid for valine 
B)  Beta 6 substitution of lysine for glutamic acid 
C)  Alpha 26 substitution of glutamic acid for threonine 
D)  Alpha 4 substitution of thymidine for methionine

Question 12

In hemoglobin C disease, how does the mutation affect the hemoglobin molecule?

Accepted Answer

A)  It cannot carry oxygen. 
B)  It has decreased hemoglobin solubility. 
C)  It is unstable. 
D)  It has increased oxygen affinity. 
A)  It cannot carry oxygen. 
B)  It has decreased hemoglobin solubility. 
C)  It is unstable. 
D)  It has increased oxygen affinity.

Question 13

Evolution dictates that patients with sickle cell disease have an inherent resistance to which of the following?

Accepted Answer

A)  Babesia infections 
B)  Acute leukemia 
C)  Hemolytic transfusion reactions 
D)  Plasmodium infections 
A)  Babesia infections 
B)  Acute leukemia 
C)  Hemolytic transfusion reactions 
D)  Plasmodium infections

Exam 13: Hemoglobinopathies: Qualitative Defects

Both hemoglobin D and G migrate with which hemoglobin on alkaline gel electrophoresis?

Patients with sickle cell disease and those with sickle cell trait differ greatly in clinical presentation. List at least two features in clinical presentation, and explain why they differ.

Electrophoresis at an acid pH using a citrate agar gel separates what hemoglobin variants that travel together on cellulose acetate at pH 8.6?

Patients with sickle cell disease suffer from vaso-occlusive crises because:

Which of the following has the highest prevalence worldwide?

Sickle cell trait is not as severe as sickle cell anemia, so why is the sickle cell trait important to diagnose?

If a patient had hemoglobin S and hemoglobin A identified by electrophoresis and a positive hemoglobin solubility test, what is the patient's most likely condition?

Explain how methemoglobin is formed and how its presence impacts oxygen delivery. Be sure to include oxygen affinity in your answer.

Which laboratory method detects hemoglobinopathies?

Clinical disease that results from a genetic mutation in one or more genes that affects hemoglobin synthesis is termed:

The defect that will produce hemoglobin C disease is:

In hemoglobin C disease, how does the mutation affect the hemoglobin molecule?

Evolution dictates that patients with sickle cell disease have an inherent resistance to which of the following?

Introduction

Cellular Homeostasis

Structure and Function of Hematopoietic Organs

Hematopoiesis

The Erythrocyte

Hemoglobin

Granulocytes and Monocytes

Lymphocytes

The Platelet

The Complete Blood Count and Peripheral Blood Smear Evaluation

Introduction to Anemia

Anemias of Disordered Iron Metabolism and Heme Synthesis

Thalassemia

Megaloblastic and Nonmegaloblastic Macrocytic Anemias

Hypoproliferative Anemias

Hemolytic Anemia: Membrane Defects

Hemolytic Anemia: Enzyme Deficiencies

Hemolytic Anemia: Immune Anemias

Hemolytic Anemia: Nonimmune Defects

Nonmalignant Disorders of Leukocytes: Granulocytes and Monocytes

Nonmalignant Lymphocyte Disorders

Introduction to Hematopoietic Neoplasms

Myeloproliferative Neoplasms

Myelodysplastic Syndromes

Acute Myeloid Leukemias

Precursor Lymphoid Neoplasms

Mature Lymphoid Neoplasms

Hematopoietic Stem Cell Transplantation

Morphologic Analysis of Body Fluids in the Hematology

Primary Hemostasis

Secondary Hemostasis and Fibrinolysis

Disorders of Primary Hemostasis

Disorders of Secondary Hemostasis

Thrombophilia

Hemostasis: Laboratory Testing and Instrumentation

Hematology Procedures

Bone Marrow Examination

Automation in Hematology

Flow Cytometry

Chromosome Analysis of Hematopoietic and Lymphoid Disorders

Molecular Analysis of Hematologic Diseases

Quality Assessment in the Hematology Laboratory

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