Question 1

What finding is present in all types of a-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 D

Question 2

Homozygous a-thalassemia (--/--)has what clinical outcome?

Accepted Answer

A) incompatible with life 
B) severe anemia 
C) mild anemia 
D) no anemia 
A) incompatible with life 
B) severe anemia 
C) mild anemia 
D) no anemia A

Question 3

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 A

Question 4

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 5

Which of the following is or are expected findings in b-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 6

Which of the following is the normal approximate percentage of hemoglobins in adults?
$\begin{array}{llll}
& \underline{\alpha}_{2} \beta_{2}& \underline{\alpha}_{2} \delta_{2}& \underline{\alpha}_{2} γ_{2} \
\text { a. } & 2 \% & 2 \% & 96 \% \
\text { b. } & 2 \% & 96 \% & 2 \% \
\text { c. } & 96 \% & 2 \% & 2 \% \
\text { d. } & 40 \% & 20 \% & 40 \%
\end{array}$

Accepted Answer

C
In adults,the approximate co

Question 7

Patients who are heterozygous for Hb Lepore have a clinical course

Accepted Answer

A) that is completely benign. 
B) similar to that of b-thalassemia minor. 
C) similar to that of a-thalassemia minor (one gene deletion). 
D) similar to that of b-thalassemia major. 
A) that is completely benign. 
B) similar to that of b-thalassemia minor. 
C) similar to that of a-thalassemia minor (one gene deletion). 
D) similar to that of b-thalassemia major.

Question 8

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

Accepted Answer

A) variable but decreased $\beta$ globin chain synthesis. 
B) no b 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 b globin chain synthesis. 
C) variable but decreased $\gamma$ chain synthesis. 
D) no gamma chain synthesis.

Question 9

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 10

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 11

Hb Barts is composed of

Accepted Answer

A) $\alpha$4. 
B) $\gamma$4. 
C) $\beta$4. 
D) $\zeta$4. 
A) $\alpha$4. 
B) $\gamma$4. 
C) $\beta$4. 
D) $\zeta$4.

Question 12

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 13

Why are infants with b-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 14

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 a chain production 
C) have excess unpaired b chains present 
D) are of Northern European ethnicity 
A) have a severe anemia 
B) have three missing genes for a chain production 
C) have excess unpaired b chains present 
D) are of Northern European ethnicity

Question 15

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 16

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

Question 17

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 18

Which of the following would be an unexpected finding in homozygous b⁰-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

Question 19

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 20

Iron overload in severe b-thalassemia (b-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.

Exam 28: Thalassemias

What finding is present in all types of a-thalassemias?

Homozygous a-thalassemia (--/--)has what clinical outcome?

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

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

Which of the following is or are expected findings in b-thalassemia minor?

Which of the following is the normal approximate percentage of hemoglobins in adults? a. 2\% 2\% 96\% b. 2\% 96\% 2\% c. 96\% 2\% 2\% d. 40\% 20\% 40\%

Patients who are heterozygous for Hb Lepore have a clinical course

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

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

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

Hb Barts is composed of

Hb A₂ and Hb F can be quantified by

Why are infants with b-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 true regarding the clinical features of thalassemias?

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

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

Which of the following would be an unexpected finding in homozygous b⁰-thalassemia?

What is the basic hemoglobin defect in the thalassemias?

Iron overload in severe b-thalassemia (b-thalassemia major)patients is primarily a consequence of

An Overview of Clinical Laboratory Hematology

Safety in the Hematology Laboratory

Blood Specimen Collection

Care and Use of the Microscope

Quality Assurance in Hematology and Hemostasis Testing

Cellular Structure and Function

Hematopoiesis

Erythrocyte Production and Destruction

Erythrocyte Metabolism and Membrane Structure and Function

Hemoglobin Metabolism

Iron Kinetics and Laboratory Assessment

Leukocyte Development, Kinetics, and Functions

Platelet Production, Structure, and Function

Manual, Semiautomated and Point-of-Care Testing in Hematology

Automated Blood Cell Analysis

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

Bone Marrow Examination

Body Fluid Analysis in the Hematology Laboratory

Anemias: Red Blood Cell Morphology and Approach to Diagnosis

Disorders of Iron Kinetics 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 Erythrocyte Destruction Nonimmune Causes

Extrinsic Defects Leading to Increased Erythrocyte Destruction Immune Causes

Hemoglobinopathies (Structural Defects in Hemoglobin)

Nonmalignant Leukocyte Disorders

Cytogenetics

Molecular Diagnostics in Hematopathology

Flow Cytometric Analysis in Hematologic Disorders

Myeloproliferative Neoplasms

Myelodysplastic Syndromes

Acute Leukemias

Mature Lymphoid Neoplasms

Normal Hemostasis and Coagulation

Hemorrhagic Disorders and Laboratory Assessment

Thrombotic Disorders and Laboratory Assessment

Thrombocytopenia and Thrombocytosis

Qualitative Disorders of Platelets and Vasculature

Laboratory Evaluation of Hemostasis

Antithrombotic Therapies and their Laboratory Assessment

Hemostasis and Coagulation Instrumentation

Pediatric and Geriatric Hematology and Hemostasis

Filters

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

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

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