Question 1

Evolution dictates that patients with sickle-cell disease have an inherent resistance to what?

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

Question 2

Use this case to answer the following questions:
A 2-year-old black male was being evaluated at the 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 was normal for his birth weight, but since then, he was 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. 
 $\begin{array}{ll}
\text { TEST } & \text { RESULTS } \
\text { WBC } & 16.6 \times 10^{9} / \mathrm{L} \
\text { RBC } & 2.53 \times 10^{12} / \mathrm{L} \
\text { HGB } & 7.5 \mathrm{gm} / \mathrm{dL} \
\text { HCT } & 21.4 \% \
\text { MCV } & 84.6 \mathrm{fl} \
\text { MCH } & 18.9 \mathrm{pg} \
\text { MCHC } & 35.0 \mathrm{~g} / \mathrm{dL} \
\text { RDW } & 18.9 \
\text { PLT } & 511 \times 10^{9} / \mathrm{L}\
\text { Differential: }\
\text { Polys } & 84 \% \
\text { Stabs } & 10 \% \
\text { Lymphs } & 5 \% \
\text { Eos } & 1 \% \
\text { NRBCs } & 12
\end{array}$
-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 3

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

Accepted Answer

A) Solubility is increased. 
B) Change in the electrophoretic mobility 
C) Increase in oxygen infinity 
D) Increase in flexibility of the red blood cell 
A) Solubility is increased. 
B) Change in the electrophoretic mobility 
C) Increase in oxygen infinity 
D) Increase in flexibility of the red blood cell

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 ability of hemoglobin 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 ability of hemoglobin 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 abnormal hemoglobin is slow-moving, and migrates closely with hemoglobin A2 on cellulose acetate at an alkaline pH?

Accepted Answer

A) Hemoglobin S 
B) Hemoglobin D 
C) Hemoglobin M 
D) Hemoglobin C 
A) Hemoglobin S 
B) Hemoglobin D 
C) Hemoglobin M 
D) Hemoglobin C

Question 6

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

Accepted Answer

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

Question 7

Choose the defect from the choices below that will produce hemoglobin C disease.

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 8

Which of the following patients would have the highest percentage of hemoglobin S?

Accepted Answer

A) A neonate with sickle-cell disease 
B) An adult with sickle-cell disease 
C) An adult with sickle-cell trait 
D) An adult with SC disease 
A) A neonate with sickle-cell disease 
B) An adult with sickle-cell disease 
C) An adult with sickle-cell trait 
D) An adult with SC disease

Question 9

Use this case to answer the following questions:
A 2-year-old black male was being evaluated at the 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 was normal for his birth weight, but since then, he was 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. 
 $\begin{array}{ll}
\text { TEST } & \text { RESULTS } \
\text { WBC } & 16.6 \times 10^{9} / \mathrm{L} \
\text { RBC } & 2.53 \times 10^{12} / \mathrm{L} \
\text { HGB } & 7.5 \mathrm{gm} / \mathrm{dL} \
\text { HCT } & 21.4 \% \
\text { MCV } & 84.6 \mathrm{fl} \
\text { MCH } & 18.9 \mathrm{pg} \
\text { MCHC } & 35.0 \mathrm{~g} / \mathrm{dL} \
\text { RDW } & 18.9 \
\text { PLT } & 511 \times 10^{9} / \mathrm{L}\
\text { Differential: }\
\text { Polys } & 84 \% \
\text { Stabs } & 10 \% \
\text { Lymphs } & 5 \% \
\text { Eos } & 1 \% \
\text { NRBCs } & 12
\end{array}$
-This patient presented with swollen hands and feet.What kind of crisis is this patient suffering from?

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 10

Use this case to answer the follwing questions:
During a discussion of RBC indices, a medical technologist of Cambodian origin mentioned that her own red cells were small in a CBC per?formed two years earlier in a routine physical. A more recent CBC showed the following results.
 $\begin{array}{ll}
\text { TEST } & \text { RESULTS } \
\text { WBC } & 9.3 \times 10^{9} / \mathrm{L} \
\text { RBC } & 6.11 \times 10^{12} / \mathrm{L} \
\mathrm{HGB} & 13.0 \mathrm{~g} / \mathrm{dL} \
\mathrm{HCT} & 41.8 \% \
\text { MCV } & 69.6 \mathrm{fL} \
\text { MCH } & 22.5 \mathrm{pg} \
\text { MCHC } & 31.0 \mathrm{~g} / \mathrm{dL} \
\mathrm{RDW} & 15.8 \
\mathrm{PLT} & 351 \times 10^{9} / \mathrm{L}\
\text { Differential }& \text { Within normal limits }\
\end{array}$
-Based on the demographics of this patient what disorder is suspected?

Accepted Answer

A) Sickle-cell disease 
B) Hemoglobin C disease 
C) Hemoglobin SC disease 
D) Hemoglobin E disease 
A) Sickle-cell disease 
B) Hemoglobin C disease 
C) Hemoglobin SC disease 
D) Hemoglobin E disease

Question 11

A patient sample is run on cellulose acetate, and two bands are found.The first band migrates to the A region on the gel.The second band migrates to the S-D-G region on the band.What is the next step to identify of the second band ?

Accepted Answer

A) Perform electrophoresis on citrate agar at pH 6.2 
B) Perform an osmotic fragility test 
C) Perform an acid elution test 
D) Do a supravital stain for Heinz bodies 
A) Perform electrophoresis on citrate agar at pH 6.2 
B) Perform an osmotic fragility test 
C) Perform an acid elution test 
D) Do a supravital stain for Heinz bodies

Question 12

The strength and type of charge on the hemoglobin molecule will enable 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 13

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

Exam 10: Hemoglobinopaties: Qualitative Defects

Evolution dictates that patients with sickle-cell disease have an inherent resistance to what?

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

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

Which abnormal hemoglobin is slow-moving, and migrates closely with hemoglobin A2 on cellulose acetate at an alkaline pH?

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

Choose the defect from the choices below that will produce hemoglobin C disease.

Which of the following patients would have the highest percentage of hemoglobin S?

A patient sample is run on cellulose acetate, and two bands are found.The first band migrates to the A region on the gel.The second band migrates to the S-D-G region on the band.What is the next step to identify of the second band ?

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

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

Introduction

Cellular Homeostasis

Hematopoiesis

Structure and Function of Hematopoietic Organs

The Erythrocyte

Hemoglobin

The Leukocyte

Introduction to Anemia

Anemias of Disordered Iron Metabolism and Heme Synthesis

Thalassemia

Megaloblastic and Nonmegaloblastic Macrocytic Anemias

Introduction to Hemolytic Anemia

Hemolytic Anemia: Membrane Defects

Hemolytic Anemia: Enzyme Deficiencies

Hemolytic Anemia: Immune Anemias

Hemolytic Anemia: Nonimmune Defects

Nonmalignant Granulocyte and Monocyte Disorders

Nonmalignant Lymphocyte Disorders

Introduction to Hematopoietic Neoplasms

Myeloproliferative Disorders

Myelodysplastic Syndromes

Acute Myeloid Leukemias

Acute Lymphoblastic Leukemias

Lymphoid Malignancies: Chronic Lymphoid Leukemias, Lymphomas, and Plasma Cell 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

Hematology Procedures and Hemostasis

Bone Marrow Examination

Automation in Hematology and Hemostasis

Flow Cytometry

Chromosome Analysis of Hematopoietic and Lymphoid Disorders

Molecular Analysis of Hematologic Diseases

Laboratory Testing in Coagulation

Quality Assessment in the Hematology Laboratory

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