Question 1

Which of the following is useful in helping to distinguish mild asymptomatic iron deficiency from thalassemia trait?

Accepted Answer

A)  Low MCV with relatively high red blood count 
B)  Mild anemia 
C)  Increased osmotic fragility 
D)  Increased ferritin 
A)  Low MCV with relatively high red blood count 
B)  Mild anemia 
C)  Increased osmotic fragility 
D)  Increased ferritin B

Question 2

Which of the following would be an unexpected finding in homozygous $\beta$0-thalassemia?

Accepted Answer

A)  Decreased reticulocyte production index 
B)  Normal Hb F 
C)  Bone marrow erythroid hyperplasia 
D)  Severe anemia 
A)  Decreased reticulocyte production index 
B)  Normal Hb F 
C)  Bone marrow erythroid hyperplasia 
D)  Severe anemia B

Question 3

Hb Barts is composed of:

Accepted Answer

A)  ($\alpha$4) 
B)  ($\gamma$4) 
C)  ($\beta$4) 
D)  ( 4) 
A)  ($\alpha$4) 
B)  ($\gamma$4) 
C)  ($\beta$4) 
D)  ( 4) B

Question 4

What finding is present in all types of $\alpha$-thalassemias?

Accepted Answer

A)  Hemoglobin H (Hb H) inclusions seen with Brilliant cresyl blue 
B)  Anemia 
C)  Microcytic hypochromic red cells 
D)  Barts hemoglobin present at birth 
A)  Hemoglobin H (Hb H) inclusions seen with Brilliant cresyl blue 
B)  Anemia 
C)  Microcytic hypochromic red cells 
D)  Barts hemoglobin present at birth

Question 5

Hemoglobin E (Hb E) is primarily found in individuals of which ethnic origin?

Accepted Answer

A)  Mediterranean 
B)  African 
C)  Southeast Asian 
D)  Northern European 
A)  Mediterranean 
B)  African 
C)  Southeast Asian 
D)  Northern European

Question 6

A patient with $\beta$+-thalassemia has, from that gene locus:

Accepted Answer

A)  Variable but decreased $\beta$globin chain synthesis 
B)  No $\beta$globin chain synthesis 
C)  Variable but decreased $\gamma$ chain synthesis 
D)  No gamma chain synthesis 
A)  Variable but decreased $\beta$globin chain synthesis 
B)  No $\beta$globin chain synthesis 
C)  Variable but decreased $\gamma$ chain synthesis 
D)  No gamma chain synthesis

Question 7

Which of the following is true regarding the clinical features of thalassemias?

Accepted Answer

A)  Are mild conditions rarely requiring treatment 
B)  Are serious conditions requiring frequent blood transfusions 
C)  Have abnormal red cells but little clinical implications for patients 
D)  Vary from benign forms to those incompatible with life 
A)  Are mild conditions rarely requiring treatment 
B)  Are serious conditions requiring frequent blood transfusions 
C)  Have abnormal red cells but little clinical implications for patients 
D)  Vary from benign forms to those incompatible with life

Question 8

Which of the following is or are expected findings in $\beta$-thalassemia minor?

Accepted Answer

A)  Basophilic stippling 
B)  Normal levels of Hb F and Hb A2 
C)  Normocytic normochromic red cells 
D)  Hemoglobin levels in the 4- to 6-g/dL range 
A)  Basophilic stippling 
B)  Normal levels of Hb F and Hb A2 
C)  Normocytic normochromic red cells 
D)  Hemoglobin levels in the 4- to 6-g/dL range

Question 9

Why are infants with$\beta$-thalassemia major not ill until approximately 6 months of age?

Accepted Answer

A)  Infants are protected by their high concentration of Hb F. 
B)  Infants have less need for hemoglobin because of their small body size. 
C)  Infants have less need for hemoglobin because of their smaller lung capacity. 
D)  Infants have a high red count and thus higher hemoglobin from a higher than normal concentration of erythropoietin. 
A)  Infants are protected by their high concentration of Hb F. 
B)  Infants have less need for hemoglobin because of their small body size. 
C)  Infants have less need for hemoglobin because of their smaller lung capacity. 
D)  Infants have a high red count and thus higher hemoglobin from a higher than normal concentration of erythropoietin.

Question 10

Which of the following is true of patients with Hb H disease?

Accepted Answer

A)  Have a severe anemia 
B)  Have three missing genes for$\alpha$chain production 
C)  Have excess unpaired $\beta$ chains present 
D)  Are of Northern European ethnicity 
A)  Have a severe anemia 
B)  Have three missing genes for$\alpha$chain production 
C)  Have excess unpaired $\beta$ chains present 
D)  Are of Northern European ethnicity

Question 11

Which of the following is least useful in diagnosing and differentiating the thalassemias from each other?

Accepted Answer

A)  Complete blood count (CB
C)  results 
B)  Hemoglobin electrophoresis 
C)  Osmotic fragility 
D)  Clinical findings 
A)  Complete blood count (CB
C)  results 
B)  Hemoglobin electrophoresis 
C)  Osmotic fragility 
D)  Clinical findings

Question 12

All of the following can distinguish $\beta$-thalassemia minor from major except:

Accepted Answer

A)  Clinical findings 
B)  Hemoglobin concentration 
C)  Presence of microcytosis, hypochromasia, and target cells 
D)  Presence of Hb A 
A)  Clinical findings 
B)  Hemoglobin concentration 
C)  Presence of microcytosis, hypochromasia, and target cells 
D)  Presence of Hb A

Question 13

Which of the following is the primary mechanism for anemia in the thalassemias?

Accepted Answer

A)  An imbalance in the rate of globin chain synthesis 
B)  Impaired iron incorporation into the protoporphyrin ring 
C)  Erythroid hypoplasia in the bone marrow 
D)  Abnormal amino acid structure of a globin chain 
A)  An imbalance in the rate of globin chain synthesis 
B)  Impaired iron incorporation into the protoporphyrin ring 
C)  Erythroid hypoplasia in the bone marrow 
D)  Abnormal amino acid structure of a globin chain

Question 14

What is the basic hemoglobin defect in the thalassemias?

Accepted Answer

A)  One of the globin chains has an amino acid substitution. 
B)  A structurally normal globin chain is absent or produced at lower levels. 
C)  Heme is produced at a lower concentration. 
D)  Iron is not incorporated into the protoporphyrin ring to form heme. 
A)  One of the globin chains has an amino acid substitution. 
B)  A structurally normal globin chain is absent or produced at lower levels. 
C)  Heme is produced at a lower concentration. 
D)  Iron is not incorporated into the protoporphyrin ring to form heme.

Question 15

Hb A2 and Hb F can be quantified by:

Accepted Answer

A)  Performing a Brilliant cresyl blue stain and counting the number of positive cells 
B)  Performing high-performance liquid chromatography 
C)  Performing cellulose acetate hemoglobin electrophoresis 
D)  Performing citrate agar hemoglobin electrophoresis 
A)  Performing a Brilliant cresyl blue stain and counting the number of positive cells 
B)  Performing high-performance liquid chromatography 
C)  Performing cellulose acetate hemoglobin electrophoresis 
D)  Performing citrate agar hemoglobin electrophoresis

Question 16

Patients who are heterozygous for Hb Lepore have a clinical course:

Accepted Answer

A)  That is completely benign 
B)  Similar to that of $\beta$-thalassemia minor 
C)  Similar to that of $\alpha$-thalassemia minor (one gene deletion) 
D)  Similar to that of $\beta$-thalassemia major 
A)  That is completely benign 
B)  Similar to that of $\beta$-thalassemia minor 
C)  Similar to that of $\alpha$-thalassemia minor (one gene deletion) 
D)  Similar to that of $\beta$-thalassemia major

Question 17

Iron overload in severe $\beta$-thalassemia ($\beta$-thalassemia major) patients is primarily a consequence of:

Accepted Answer

A)  An increased rate of absorption of iron because of the severe anemia 
B)  A decreased need for iron because fewer RBCs are made 
C)  Accumulation of iron from massive RBC transfusion therapy 
D)  Decreased rate of use of iron because smaller RBCs are made 
A)  An increased rate of absorption of iron because of the severe anemia 
B)  A decreased need for iron because fewer RBCs are made 
C)  Accumulation of iron from massive RBC transfusion therapy 
D)  Decreased rate of use of iron because smaller RBCs are made

Question 18

A patient has 30% Hb F. Which of the following can be eliminated?

Accepted Answer

A)  Four missing genes for $\alpha$ chain production 
B)  Heterozygous hereditary persistence of Hb F 
C)  Thalassemia intermedia 
D)  The patient is a normal 1-month-old infant 
A)  Four missing genes for $\alpha$ chain production 
B)  Heterozygous hereditary persistence of Hb F 
C)  Thalassemia intermedia 
D)  The patient is a normal 1-month-old infant

Question 19

Which of the following can help distinguish the combination Hemoglobin S (Hb S) $\beta$0-thalassemia from sickle cell anemia?

Accepted Answer

A)  Hematocrit 
B)  Hemoglobin electrophoresis 
C)  Ethnic background 
D)  Mean cell volume (MCV) 
A)  Hematocrit 
B)  Hemoglobin electrophoresis 
C)  Ethnic background 
D)  Mean cell volume (MCV)

Question 20

Which of the following is the normal approximate percentage of hemoglobins in adults? $\alpha$2$\beta$2 $\alpha$2$\delta$ 2 $\alpha$2$\gamma$2

Accepted Answer

A)  2% 2% 96% 
B)  2% 96% 2% 
C)  96% 2% 2% 
D)  40% 20% 40% 
A)  2% 2% 96% 
B)  2% 96% 2% 
C)  96% 2% 2% 
D)  40% 20% 40%

Exam 27: Thalassemias

Which of the following is useful in helping to distinguish mild asymptomatic iron deficiency from thalassemia trait?

Which of the following would be an unexpected finding in homozygous β\betaβ 0-thalassemia?

Hb Barts is composed of:

What finding is present in all types of α\alphaα -thalassemias?

Hemoglobin E (Hb E) is primarily found in individuals of which ethnic origin?

A patient with β\betaβ +-thalassemia has, from that gene locus:

Which of the following is true regarding the clinical features of thalassemias?

Which of the following is or are expected findings in β\betaβ -thalassemia minor?

Why are infants with β\betaβ -thalassemia major not ill until approximately 6 months of age?

Which of the following is true of patients with Hb H disease?

Which of the following is least useful in diagnosing and differentiating the thalassemias from each other?

All of the following can distinguish β\betaβ -thalassemia minor from major except:

Which of the following is the primary mechanism for anemia in the thalassemias?

What is the basic hemoglobin defect in the thalassemias?

Hb A2 and Hb F can be quantified by:

Patients who are heterozygous for Hb Lepore have a clinical course:

Iron overload in severe β\betaβ -thalassemia ( β\betaβ -thalassemia major) patients is primarily a consequence of:

A patient has 30% Hb F. Which of the following can be eliminated?

Which of the following can help distinguish the combination Hemoglobin S (Hb S) β\betaβ 0-thalassemia from sickle cell anemia?

Which of the following is the normal approximate percentage of hemoglobins in adults? α\alphaα 2 β\betaβ 2 α\alphaα 2 δ\deltaδ 2 α\alphaα 2 γ\gammaγ 2

An Overview of Clinical Laboratory Hematology

Safety in the Hematology Laboratory

Specimen Collection

Care and Use of the Microscope

Quality Assurance in Hematology and Hemostasis Testing

Cellular Structure and Function

Hematopoiesis

Erythrocyte Production and Destruction

Energy Metabolism and Membrane Physiology of the Erythrocyte

Hemoglobin Metabolism

Iron Metabolism

Leukocyte Development, Kinetics, and Functions

Platelet Production, Structure, and Function

Routine and Point-Of-Care Testing in Hematology: Manual and Semiautomated Methods

Examination of the Peripheral Blood Film and Correlation With the Complete Blood Count

Bone Marrow Examination

Body Fluids in the Hematology Laboratory

Anemias: Red Blood Cell Morphology and Approach to Diagnosis

Disorders of Iron and Heme Metabolism

Anemias Caused by Defects of Dna Metabolism

Bone Marrow Failure

Introduction to Increased Destruction of Erythrocytes

Intrinsic Defects Leading to Increased Erythrocyte Destruction

Extrinsic Defects Leading to Increased Erythrocytedestructionnonimmune Causes

Extrinsic Defects Leading to Increased Erythrocytedestructionimmune Causes

Hemoglobinopathies Structural Defects in Hemoglobin

Nonmalignant Leukocyte Disorders

Introduction to Leukocyte Neoplasms

Cytochemistry

Cytogenetics

Molecular Diagnostics in the Clinical Laboratory

Flow Cytometric Analysis in Hematologic Disorders

Myeloproliferative Neoplasms

Myelodysplastic Syndromes

Acute Leukemias

Mature Lymphoid Neoplasms

Pediatric and Geriatric Hematology

Automated Cell-Counting Instrumentation

Normal Hemostasis and Coagulation

Hemorrhagic Coagulation Disorders

Thrombosis Risk Testing

Thrombocytopenia and Thrombocytosis

Qualitative Disorders of Platelets and Vasculature

Laboratory Evaluation of Hemostasis

Monitoring Antithrombotic Therapies

Coagulation Instrumentation

Filters

Which of the following would be an unexpected finding in homozygous $\beta$ ⁰-thalassemia?

What finding is present in all types of $\alpha$ -thalassemias?

A patient with $\beta$ ⁺-thalassemia has, from that gene locus:

Which of the following is or are expected findings in $\beta$ -thalassemia minor?

Why are infants with $\beta$ -thalassemia major not ill until approximately 6 months of age?

All of the following can distinguish $\beta$ -thalassemia minor from major except:

Hb A₂ and Hb F can be quantified by:

Iron overload in severe $\beta$ -thalassemia ( $\beta$ -thalassemia major) patients is primarily a consequence of:

Which of the following can help distinguish the combination Hemoglobin S (Hb S) $\beta$ ⁰-thalassemia from sickle cell anemia?

Which of the following is the normal approximate percentage of hemoglobins in adults? $\alpha$ ₂ $\beta$ ₂ $\alpha$ ₂ $\delta$ ₂ $\alpha$ ₂ $\gamma$ ₂