Question 1

Disease that results from a molecular defect in hemoglobin synthesis in RBCs is termed:

Accepted Answer

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

Question 2

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 D

Question 3

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 to the health care provider because of symptoms related to vaso-occlusive crises.These include tingling fingers and toes,cyanosis,shortness of breath,and joint pain.Patients with sickle cell trait are often asymptomatic and learn of their condition as a result of routine blood examination.

Question 4

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 5

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 6

Other than hemoglobin S,name two abnormal hemoglobins that produce a positive sickle solubility test.Explain how they could be differentiated from HbS.

Accepted Answer

Hemoglobins C-Harlem and HbI have positi

Question 7

How is methemoglobin detected in the laboratory?

Accepted Answer

Spectral analysis at

Question 8

Use this case to answer the following questions:
A 2-year-old black male was being evaluated at County Clinic for swollen hands and feet that were extremely painful.Upon physical examination,the child was found to have a fever and hepatosplenomegaly and was physically small for his age.The child was of Jamaican descent.
Patient history revealed that the child's birth weight was normal,but since then,he had been consistently underweight and undersized for his age group.Family history was not readily available.The physician had the child admitted to the hospital for workup and ordered the following CBC.  
-Based on these findings,what test would be the logical next step?

Accepted Answer

A) Bone marrow aspirate 
B) Osmotic fragility 
C) Sickle solubility test 
D) Peripheral blood smear 
A) Bone marrow aspirate 
B) Osmotic fragility 
C) Sickle solubility test 
D) Peripheral blood smear

Question 9

Thalassemias are produced as a result of:

Accepted Answer

A) Quantitative defects in globin chain synthesis 
B) Qualitative defects in globin chain synthesis 
C) Structural defects in heme synthesis 
D) Molecular defects in hemoglobin synthesis 
A) Quantitative defects in globin chain synthesis 
B) Qualitative defects in globin chain synthesis 
C) Structural defects in heme synthesis 
D) Molecular defects in hemoglobin synthesis

Question 10

Use this case to answer the following questions:
A 2-year-old black male was being evaluated at County Clinic for swollen hands and feet that were extremely painful.Upon physical examination,the child was found to have a fever and hepatosplenomegaly and was physically small for his age.The child was of Jamaican descent.
Patient history revealed that the child's birth weight was normal,but since then,he had been consistently underweight and undersized for his age group.Family history was not readily available.The physician had the child admitted to the hospital for workup and ordered the following CBC.  
-All of the following parameters of the complete blood count are abnormal except:

Accepted Answer

A) Hemoglobin 
B) MCV 
C) RBCs 
D) Platelet count 
A) Hemoglobin 
B) MCV 
C) RBCs 
D) Platelet count

Question 11

The net decrease in negative charge in the hemoglobin S molecule is responsible for which laboratory finding?

Accepted Answer

A) Increase in solubility 
B) Change in the electrophoretic mobility 
C) Increase in oxygen infinity 
D) Increase in flexibility of the red blood cell 
A) Increase in solubility 
B) Change in the electrophoretic mobility 
C) Increase in oxygen infinity 
D) Increase in flexibility of the red blood cell

Question 12

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 13

In hemoglobin C disease,the cell's life span is decreased to what length?

Accepted Answer

A) 90-120 days 
B) 7-10 days 
C) 75-90 days 
D) 30-55 days 
A) 90-120 days 
B) 7-10 days 
C) 75-90 days 
D) 30-55 days

Question 14

Use this case to answer the following questions:
A 2-year-old black male was being evaluated at County Clinic for swollen hands and feet that were extremely painful.Upon physical examination,the child was found to have a fever and hepatosplenomegaly and was physically small for his age.The child was of Jamaican descent.
Patient history revealed that the child's birth weight was normal,but since then,he had been consistently underweight and undersized for his age group.Family history was not readily available.The physician had the child admitted to the hospital for workup and ordered the following CBC.  
-This patient presented with swollen hands and feet.This patient suffering from what kind of crisis?

Accepted Answer

A) Vaso-occlusive 
B) Oxidative 
C) Transfusion reaction 
D) Anaphylactic reaction 
A) Vaso-occlusive 
B) Oxidative 
C) Transfusion reaction 
D) Anaphylactic reaction

Question 15

The strength and type of charge on a hemoglobin molecule enables the separation of hemoglobin variants by electrophoresis.What other significant factor controls this separation?

Accepted Answer

A) Solubility of the molecule 
B) pH of the medium 
C) Heat precipitation 
D) Elevation of hemoglobin concentration 
A) Solubility of the molecule 
B) pH of the medium 
C) Heat precipitation 
D) Elevation of hemoglobin concentration

Question 16

The defect in the following choices that will produce hemoglobin C disease are:

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 17

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 18

What preoperative therapy is effective in a diagnosed sickle cell patient?

Accepted Answer

A) Stem cell transplant 
B) Transfusion of red blood cells 
C) Hydroxyurea administration 
D) Gene therapy 
A) Stem cell transplant 
B) Transfusion of red blood cells 
C) Hydroxyurea administration 
D) Gene therapy

Question 19

Hemoglobin C disease has altered solubility properties similar to hemoglobin S disease.What accounts for this altered solubility?

Accepted Answer

A) Heme iron is in the ferric form rather than ferrous form. 
B) The hemoglobin remains in the tense structure. 
C) A nonpolar amino acid is substituted for a polar amino acid. 
D) A polar amino acid is substituted near the pocket where heme binds. 
A) Heme iron is in the ferric form rather than ferrous form. 
B) The hemoglobin remains in the tense structure. 
C) A nonpolar amino acid is substituted for a polar amino acid. 
D) A polar amino acid is substituted near the pocket where heme binds.

Question 20

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.

Exam 13: Hemoglobinopathies: Qualitative Defects

Disease that results from a molecular defect in hemoglobin synthesis in RBCs is termed:

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

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.

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?

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

Other than hemoglobin S,name two abnormal hemoglobins that produce a positive sickle solubility test.Explain how they could be differentiated from HbS.

How is methemoglobin detected in the laboratory?

Thalassemias are produced as a result of:

The net decrease in negative charge in the hemoglobin S molecule is responsible for which laboratory finding?

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

In hemoglobin C disease,the cell's life span is decreased to what length?

The strength and type of charge on a hemoglobin molecule enables the separation of hemoglobin variants by electrophoresis.What other significant factor controls this separation?

The defect in the following choices that will produce hemoglobin C disease are:

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

What preoperative therapy is effective in a diagnosed sickle cell patient?

Hemoglobin C disease has altered solubility properties similar to hemoglobin S disease.What accounts for this altered solubility?

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

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 Laboratory

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

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