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MULTIPLE CHOICE
1. What is the cause of polycythemia in the fetus?
a. Fetal hemoglobin has a greater affinity for oxygen as a result of diphosphoglycerate
(DPG).
b. The fetus has a different hemoglobin structure of two - and two -chains rather
environment.
d. The lungs of the fetus are undeveloped and unable to diffuse oxygen adequately to
The hypoxic intrauterine environment stimulates erythropoietin production in the fetus. This
selection is the only option that accurately explains why the fetus develops polycythemia.
PTS: 1
2. Why does fetal hemoglobin have a greater affinity for oxygen than adult hemoglobin?
a. The fetus does not have its own oxygen supply and must rely on oxygen from the
adult.
c. Fetal hemoglobin interacts less readily with diphosphoglycerate (DPG), which
Fetal hemoglobin has greater affinity for oxygen than does adult hemoglobin because it
interacts less readily with the enzyme, DPG, which inhibits hemoglobin-oxygen binding. This
selection is the only option that accurately explains why fetal hemoglobin has a greater
affinity for oxygen than adult hemoglobin.
PTS: 1
3. Which blood cell type is elevated at birth but decreases to adult levels during the first year of
life?
a. Monocytes
b. Platelets
c. Neutrophils
d. Lymphocytes
ANS: A
Only monocyte counts are high in the first year of life and then decrease to adult levels.
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4. In a full-term infant, the normal erythrocyte life span is _____ days, whereas the adult
a. 30 to 50; 80
b. 60 to 80; 120
c. 90 to 110; 140
d. 120 to 130; 150
ANS: B
In full-term infants, normal erythrocyte life span is 60 to 80 days; in premature infants it may
be as short as 20 to 30 days; and in children and adolescents, it is 120 days, the same as that in
adults.
PTS: 1
6. How does hemolytic disease of the newborn (HDN) cause acquired congenital hemolytic
anemia?
a. HDN develops when hypoxia or dehydration causes the erythrocytes to change
shapes, which are then recognized as foreign and removed from circulation.
b. HDN is an alloimmune disease in which the mothers immune system produces
antibodies against fetal erythrocytes, which are recognized as foreign and removed
from circulation.
c. HDN develops when the polycythemia present in fetal life continues after birth,
causing the excessive number of erythrocytes to be removed from circulation.
d. HDN is an autoimmune disease in which the fetuss immune system produces
antibodies against fetal erythrocytes, which are recognized as foreign and removed
from circulation.
ANS: B
HDN is an alloimmune disease in which maternal blood and fetal blood are antigenically
incompatible, causing the mothers immune system to produce antibodies against fetal
erythrocytes. Fetal erythrocytes that have been attacked by (or bound to) maternal antibodies
are recognized as foreign or defective by the fetal mononuclear phagocyte system and are
removed from the circulation by phagocytosis, usually in the fetal spleen. This selection is the
only option that accurately explains how HDN causes acquired congenital hemolytic anemia.
PTS: 1
incompatible
b. Alloimmune disease in which maternal blood and fetal blood are antigenically
incompatible
c. Autoimmune disease in immature nucleated cells that are released into the
bloodstream
8. An infants hemoglobin must fall below ___ g/dl before signs of pallor, tachycardia, and
c. 7
d. 5
ANS: D
When the hemoglobin falls below 5 g/dl, pallor, tachycardia, and systolic murmurs may occur.
PTS: 1
9. Which vitamin improves the absorption of oral iron taken to treat iron deficiency anemia in
children?
a. A
b. B
c. C
d. E
ANS: C
Of the available options, only additional vitamin C may be used to promote iron absorption.
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10. Hemolytic disease of the newborn (HDN) can occur if the mother:
a. Is Rh-positive and the fetus is Rh-negative
b. Is Rh-negative and the fetus is Rh-positive
c. Has type A blood and the fetus has type O
d. Has type AB blood and the fetus has type B
ANS: B
HDN can occur only if antigens on fetal erythrocytes differ from antigens on maternal
erythrocytes. Maternal-fetal incompatibility exists only if the mother and fetus differ in ABO
blood type or if the fetus is Rh-positive and the mother is Rh-negative. This erythrocyte
incompatibility does not exist in any of the other options.
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11. When diagnosed with hemolytic disease of the newborn (HDN), why does the newborn
ANS: A
12. Fetuses who do not survive anemia in utero are usually stillborn with gross edema of the
a. Spherocytosis
b. Icterus gravis neonatorum
ANS: D
Fetuses that do not survive anemia in utero are usually stillborn with gross edema in the entire
body, a condition called hydrops fetalis. This selection is the only option that accurately
identifies the condition with these specific symptoms.
PTS: 1
13. What is the name of the disorder in which levels of bilirubin remain excessively high in the
c. Jaundice
d. Icterus gravis neonatorum
ANS: A
Without replacement transfusions, during which the child receives Rh-negative erythrocytes,
the bilirubin is deposited in the brain, a condition called kernicterus. This selection is the only
option that accurately identifies the condition with the provided symptoms.
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14. What treatment prevents the development of kernicterus in an infant born with hemolytic
anti-Rh antibodies
c. Performance of a splenectomy to prevent the destruction of abnormal erythrocytes
d. Replacement transfusion of Rh-negative erythrocytes
ANS: B
15. Glucose 6-phosphate dehydrogenase (G6PD) deficiency is what type of inherited disorder?
a. X-linked dominant
b. X-linked recessive
c. Autosomal dominant
d. Autosomal recessive
ANS: B
G6PD deficiency is an inherited, X-linked recessive disorder. This selection is the only option
that accurately identifies the mode of inheritance for the stated disorder.
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17. Hemoglobin S (HbS) is formed in sickle cell disease as a result of which process?
a. Deficiency in glucose 6-phosphate dehydrogenase (G6PD) that changes
acid).
d. Autoimmune response in which one amino acid (proline) is detected as an antigen
HbS is formed by a genetic mutation in which one amino acid (valine) replaces another
(glutamic acid) (see Figure 30-5, A). This selection is the only option that accurately identifies
how HbS is formed in sickle cell disease.
PTS: 1
SCD is an inherited autosomal recessive disorder that is expressed as sickle cell anemia, sickle
cellthalassemia disease, or sickle cellhemoglobin C (HbC) disease, depending on mode of
inheritance. This selection is the only option that accurately identifies the mode of inheritance
for the stated disorder.
PTS: 1
19. What is the reason most children diagnosed with sickle cell anemia are not candidates for
medications.
d. Although effective for adults, neither procedure has been proven effective for
children.
ANS: A
Bone marrow or stem cell transplants can cure sickle cell anemia. However, the only reason
these procedures are not currently an option for most children is because well-matched stem
cell donors are often difficult to find.
PTS: 1
20. Which manifestations of vasoocclusive crisis are associated with sickle cell disease (SCD) in
infants?
a. Atelectasis and pneumonia
b. Edema of the hands and feet
c. Stasis ulcers of the hands, ankles, and feet
d. Splenomegaly and hepatomegaly
ANS: B
Symmetric, painful swelling of the hands and feet (hand-foot syndrome) caused by infarction
in the small vessels of the extremities is often the initial manifestation of SCD in infants. This
selection is the only option that accurately identifies the manifestations of a vasoocclusive
crisis associated with SCD in infancy.
PTS: 1
21. What is the chance with each pregnancy that a child born to two parents with the sickle trait
c. 33%
d. 50%
ANS: B
A 25% chance exists with each pregnancy that a child born to two parents with sickle cell trait
will have SCD. Genetic counseling enables people with SCD or with the sickle cell trait to
make informed decisions about transmitting this genetic disorder to their offspring.
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The only option that identifies the anemic manifestation of thalassemia is microcytichypochromic hemolytic anemia.
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The fundamental defect in beta-thalassemia is the uncoupling of - and -chain synthesis. Chain production is moderately depressed in the heterozygous formbeta-thalassemia minor,
and severely depressed in the homozygous formbeta-thalassemia major (also called Cooley
anemia). The defects described in the other options are not related to beta-thalassemia major.
PTS: 1
24. The alpha- and beta-thalassemias are considered what types of inherited disorder?
a. Autosomal recessive
c. X-linked recessive
b. Autosomal dominant
d. X-linked dominant
ANS: A
The alpha- and beta-thalassemias are inherited autosomal recessive disorders. This selection is
the only option that correctly identifies how these disorders are inherited.
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The bleeding disorder, von Willebrand disease, results only from an inherited autosomal
dominant trait.
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c. Platelets
d. Basophils
ANS: C
ITP is a disorder of platelet consumption. This selection is the only option that correctly
identifies which type of cell is attacked by ITP.
PTS: 1
29. Which disorder results in decreased erythrocytes and platelets with changes in leukocytes and
has clinical manifestations of pallor, fatigue, petechiae, purpura, bleeding, and fever?
a. Idiopathic thrombocytopenic purpura (ITP)
b. Acute lymphocytic leukemia (ALL)
c. Non-Hodgkin lymphoma (NHL)
d. Iron deficiency anemia (IDA)
ANS: B
Pallor, fatigue, petechiae, purpura, bleeding, and fever are generally present with the most
common symptoms reflecting the consequence of bone marrow failure, which results in
decreased red blood cells and platelets and changes in white blood cells. This selection is the
only option that correctly identifies the disease with the symptoms described.
PTS: 1
30. When does fetal erythrocyte production shift from the liver to the bone marrow?
a. Fourth month of gestation
c. Eighth month of gestation
b. Fifth month of gestation
d. At birth
ANS: B
By the fifth month of gestation, hematopoiesis begins to occur in the bone marrow and
increases rapidly until hematopoietic (red) marrow fills the entire bone marrow space. By the
time of delivery, the bone marrow is the only significant site of hematopoiesis.
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31. Which disease is caused by clotting factor VIII deficiency and is an autosomal dominant trait?
a. Hemophilia A
c. Hemophilia C
b. Hemophilia B
d. von Willebrand disease
ANS: D
Of the options available, only von Willebrand disease is caused by factor VIII deficiency and
is the result of an inherited autosomal dominant trait.
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Of the options available, only hemophilia A (classic hemophilia) is caused by factor VIII
deficiency and is inherited as an X-linked recessive disorder that affects men and is
transmitted by women.
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Iron deficiency anemia is the most common blood disorder of infancy and childhood, with the
highest incidence occurring between 6 months and 2 years of age.
PTS: 1
35. What is the significance of hyperdiploidy when diagnosing and treating leukemia?
a. Hyperdiploidy indicates a good prognosis.
b. Hyperdiploidy indicates a poor prognosis.
c. Hyperdiploidy indicates poor response to a specific treatment.
d. Hyperdiploidy indicates the achievement of remission.
ANS: A
MULTIPLE RESPONSE
36. What are the common triggers for sickle cell crisis? (Select all that apply.)
a. Fever
b. Infection
c. Dehydration
d. Alkalosis
e. Exposure to the cold
ANS: A, B, C, E
MATCHING
Match each sickle cell crisis with its description. Terms may be used more than once.
______ A. Vasoocclusive crisis
______ B. Aplastic crisis
______ C. Sequestration crisis
______ D. Hyperhemolytic crisis
37.
38.
39.
40.
Large amounts of blood become acutely pooled in the liver and spleen.
Blood flow is impaired by tangled masses of rigid, sickled cells.
Crisis occurs in association with certain drugs or infection.
Compensatory erythropoiesis is compromised, thus limiting the number of erythrocytes that
are replaced.
37. ANS: C
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REF: Page 1066
MSC: In sequestration crisis, large amounts of blood become acutely pooled in the liver and spleen.
38. ANS: A
PTS: 1
REF: Page 1066
MSC: Vasoocclusive crisis (thrombotic crisis) begins with sickling in the microcirculation. As blood
flow is obstructed by tangled masses of rigid, sickled cells, vasospasm occurs and a log jam effect
brings all blood flow through the vessel to a halt.
39. ANS: D
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REF: Page 1066
40. ANS: B
PTS: 1
REF: Page 1066
MSC: Aplastic crisis, a transient cessation in red blood cell production that results in acute anemia,
occurs as a result of a viral infection. The virus causes a temporary shutdown of red blood cell
production in the bone marrow, or reticulocytosis.