Question 1

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 reactionE) None of the above
F) B) and C)

Question 2

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 3

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 infectionsE) All of the above
F) C) and D) D

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 traitE) A) and B)
F) All of the above

Question 5

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 synthesisE) All of the above
F) A) and D)

Question 6

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 cellE) B) and C)
F) C) and D)

Question 7

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

Accepted Answer

A)  S
B)  A
C)  F
D)  CE) A) and B)
F) B) and D)

Question 8

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.E) A) and B)
F) B) and C)

Question 9

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 daysE) A) and D)
F) A) and C)

Question 10

How is methemoglobin detected in the laboratory?

Accepted Answer

Spectral analysis at...

Question 11

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

Accepted Answer

A)  Thalassemia
B)  Hemoglobinopathy
C)  Porphyria
D)  AsynchronyE) None of the above
F) B) and D) B

Question 12

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 methionineE) C) and D)
F) A) and B)

Question 13

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 AE) A) and B)
F) All of the above

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. 
-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 smearE) All of the above
F) None of the above

Question 15

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.E) None of the above
F) B) and D)

Question 16

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 concentrationE) None of the above
F) B) and D)

Question 17

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 countE) All of the above
F) C) and D)

Question 18

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.E) All of the above
F) None of the above

Question 19

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 20

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 therapyE) None of the above
F) All of the above

Exam 13: Hemoglobinopathies: Qualitative Defects

Correct Answer
verified

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

Correct Answer
verified
Hemoglobins C-Harlem and HbI have positi...
View Answer

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

Correct Answer
verified
D

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?

Correct Answer
verified

Thalassemias are produced as a result of:

Correct Answer
verified

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

Correct Answer
verified

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

Correct Answer
verified

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

Correct Answer
verified

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

Correct Answer
verified

How is methemoglobin detected in the laboratory?

Correct Answer
verified
Spectral analysis at...
View Answer

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

Correct Answer
verified
B

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

Correct Answer
verified

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

Correct Answer
verified

Correct Answer
verified

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

Correct Answer
verified

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?

Correct Answer
verified

Correct Answer
verified

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

Correct Answer
verified

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.

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

Correct Answer
verified

Exam 13: Hemoglobinopathies: Qualitative Defects

Correct AnswerverifiedShow Answer

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

Correct AnswerverifiedHemoglobins C-Harlem and HbI have positi...View AnswerShow Answer

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

Correct AnswerverifiedD

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?

Correct AnswerverifiedShow Answer

Thalassemias are produced as a result of:

Correct AnswerverifiedShow Answer

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

Correct AnswerverifiedShow Answer

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

Correct AnswerverifiedShow Answer

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

Correct AnswerverifiedShow Answer

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

Correct AnswerverifiedShow Answer

How is methemoglobin detected in the laboratory?

Correct AnswerverifiedSpectral analysis at...View AnswerShow Answer

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

Correct AnswerverifiedB

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

Correct AnswerverifiedShow Answer

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

Correct AnswerverifiedShow Answer

Correct AnswerverifiedShow Answer

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

Correct AnswerverifiedShow Answer

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?

Correct AnswerverifiedShow Answer

Correct AnswerverifiedShow Answer

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

Correct AnswerverifiedShow Answer

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.

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

Correct AnswerverifiedShow Answer

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Hemoglobins C-Harlem and HbI have positi...
View Answer

Correct Answer
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D

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Correct Answer
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Correct Answer
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Spectral analysis at...
View Answer

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B

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Correct Answer
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Correct Answer
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