Question 1

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 2

Use this case to answer the following questions:
During a discussion of RBC indices,a medical technologist of Cambodian origin mentioned that her own red cells had been small in a CBC performed 2 years earlier in a routine physical.A more recent CBC showed the following results.  
-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 3

A patient has a positive sickle solubility test.Further investigation reveals hemoglobin of 8.5 g/dL.What can be done to confirm these findings?
 Explain your answer.

Accepted Answer

The positive sickle solubility test can

Question 4

The peripheral blood smear contains a normocytic,normochromic morphology with marked reticulocytosis.The hemoglobin solubility test is positive.What confirmatory test should follow?

Accepted Answer

A) Bilirubin analysis 
B) Haptoglobin analysis 
C) Hemoglobin electrophoresis 
D) Bone marrow aspiration 
A) Bilirubin analysis 
B) Haptoglobin analysis 
C) Hemoglobin electrophoresis 
D) Bone marrow aspiration

Question 5

What is the purpose of treating sickle cell patients with hydroxyurea?

Accepted Answer

A) To dilute the amount of sickled red blood cells 
B) To destroy the sickling red blood cells by initiating an immune response 
C) To convert Hgb S into Hgb A 
D) To increase the level of hemoglobin F that will reduce intracellular sickling 
A) To dilute the amount of sickled red blood cells 
B) To destroy the sickling red blood cells by initiating an immune response 
C) To convert Hgb S into Hgb A 
D) To increase the level of hemoglobin F that will reduce intracellular sickling

Question 6

A patient's 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 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 7

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

Accepted Answer

A) It cannot carry oxygen. 
B) It forms insoluble aggregates. 
C) It is unstable. 
D) It has increased oxygen affinity. 
A) It cannot carry oxygen. 
B) It forms insoluble aggregates. 
C) It is unstable. 
D) It has increased oxygen affinity.

Question 8

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 9

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 10

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 11

Hemoglobinopathy results from a genetic abnormality of which structure within the hemoglobin molecule?

Accepted Answer

A) Globin chain 
B) Heme 
C) Porphyrin 
D) Iron 
A) Globin chain 
B) Heme 
C) Porphyrin 
D) Iron

Question 12

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 13

Use this case to answer the following questions:
During a discussion of RBC indices,a medical technologist of Cambodian origin mentioned that her own red cells had been small in a CBC performed 2 years earlier in a routine physical.A more recent CBC showed the following results.  
-What reflex test is most appropriate for investigating these results?

Accepted Answer

A) Hemoglobin electrophoresis and iron studies 
B) Bone marrow aspirate 
C) Vitamin B₁₂ and folate assays 
D) Cytogenetic analysis 
A) Hemoglobin electrophoresis and iron studies 
B) Bone marrow aspirate 
C) Vitamin B₁₂ and folate assays 
D) Cytogenetic analysis

Exam 13: Hemoglobinopathies: Qualitative Defects

Which of the following has the highest prevalence worldwide?

A patient has a positive sickle solubility test.Further investigation reveals hemoglobin of 8.5 g/dL.What can be done to confirm these findings? Explain your answer.

The peripheral blood smear contains a normocytic,normochromic morphology with marked reticulocytosis.The hemoglobin solubility test is positive.What confirmatory test should follow?

What is the purpose of treating sickle cell patients with hydroxyurea?

A patient's 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 the second band?

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

Which laboratory method detects hemoglobinopathies?

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

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

Hemoglobinopathy results from a genetic abnormality of which structure within the hemoglobin molecule?

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

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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