Chapter 30: Alterations of Hematologic Function in Children

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

than two -and two -chains.

c. Increased erythropoiesis occurs in response to the hypoxic intrauterine

environment.
d. The lungs of the fetus are undeveloped and unable to diffuse oxygen adequately to

the pulmonary capillaries.

ANS: C

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

REF: Page 1057

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

maternal vascular system.

b. The fetus has two -chains on the hemoglobin, rather than two -chains as in the

adult.
c. Fetal hemoglobin interacts less readily with diphosphoglycerate (DPG), which

inhibits hemoglobin-oxygen binding.

d. Fetal hemoglobin production occurs in the vessels and liver rather than in the bone

marrow as in the adult.

ANS: C

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

REF: Page 1056

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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REF: Page 1057

4. In a full-term infant, the normal erythrocyte life span is _____ days, whereas the adult

erythrocyte life span is _____ days.

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.
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REF: Page 1057

5. What is the most common cause of insufficient erythropoiesis in children?

a. Folic acid deficiency
c. Hemoglobin abnormality
b. Iron deficiency
d. Erythrocyte abnormality
ANS: B

Similar to the anemias of adulthood, ineffective erythropoiesis or premature destruction of

erythrocytes causes the anemias of childhood. The most common cause of insufficient
erythropoiesis is iron deficiency. The other options may be causes but are not common ones.
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REF: Page 1058

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.
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REF: Page 1058

7. Erythroblastosis fetalis is defined as an:

a. Allergic disease in which maternal blood and fetal blood are antigenically

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

d. Autosomal dominant hereditary disease

ANS: B

Erythroblastosis fetalis, also known as hemolytic disease of the newborn (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. This
selection is the only option that accurately defines erythroblastosis fetalis.
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REF: Page 1058

8. An infants hemoglobin must fall below ___ g/dl before signs of pallor, tachycardia, and

systolic murmurs occur.

a. 11
b. 9

c. 7
d. 5

ANS: D

When the hemoglobin falls below 5 g/dl, pallor, tachycardia, and systolic murmurs may occur.
PTS: 1

REF: Page 1059

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.
PTS: 1

REF: Page 1059

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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REF: Page 1059

11. When diagnosed with hemolytic disease of the newborn (HDN), why does the newborn

develop hyperbilirubinemia after birth but not in utero?

a. Excretion of unconjugated bilirubin through the placenta into the mothers
circulation is no longer possible.
b. Hemoglobin does not break down into bilirubin in the intrauterine environment.
c. The liver of the fetus is too immature to conjugate bilirubin from a lipid-soluble to
water-soluble form.
d. The destruction of erythrocytes producing bilirubin is greater after birth.

ANS: A

Hyperbilirubinemia occurs in the neonate after birth because excretion of lipid-soluble

unconjugated bilirubin through the placenta is no longer possible. This selection is the only
option that accurately explains why HDN causes hyperbilirubinemia after birth but not in
utero.
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REF: Page 1060

12. Fetuses who do not survive anemia in utero are usually stillborn with gross edema of the

entire body. Which term is used to identify this condition?

c. Erythroblastosis fetalis
d. Hydrops fetalis

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.
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REF: Page 1060

13. What is the name of the disorder in which levels of bilirubin remain excessively high in the

newborn and are deposited in the brain?

a. Kernicterus
b. Icterus neonatorum

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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REF: Page 1060

14. What treatment prevents the development of kernicterus in an infant born with hemolytic

disease of the newborn (HDN)?

a. Administration of intravenous fluids to dilute the blood
b. Replacement transfusion of new Rh-positive blood that is not contaminated with

anti-Rh antibodies
c. Performance of a splenectomy to prevent the destruction of abnormal erythrocytes
d. Replacement transfusion of Rh-negative erythrocytes
ANS: B

If antigenic incompatibility of the mothers erythrocytes is not discovered in time to

administer Rh immunoglobulin and the child is born with HDN, then the treatment consists of
exchange transfusions in which the neonates blood is replaced with new Rh-positive blood
that is not contaminated with anti-Rh antibodies. This treatment is administered during the
first 24 hours of extrauterine life to prevent kernicterus. This selection is the only option that
accurately prevents kernicterus.
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REF: Page 1061

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.
PTS: 1

REF: Page 1062

16. Sickle cell disease is classified as a(an):

a. Inherited X-linked recessive disorder
b. Inherited autosomal recessive disorder
c. Disorder initiated by hypoxemia and acidosis
d. Disorder that is diagnosed equally in men and women
ANS: C

Sickling is an occasional, intermittent phenomenon that can be triggered or sustained by one

or more of the following stressors: decreased oxygen tension (PO2) of the blood (hypoxemia),
increased hydrogen ion concentration in the blood (decreased pH), increased plasma
osmolality, decreased plasma volume, and low temperature (see Figure 30-7). This selection is
the only option that accurately identifies the classification of the stated disorders.
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REF: Page 1062 | Page 1065

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

hemoglobin A (HbA) to HbS.

b. Genetic mutation in which two amino acids (histidine and leucine) are missing.
c. Genetic mutation in which one amino acid (valine) is replaced by another (glutamic

acid).
d. Autoimmune response in which one amino acid (proline) is detected as an antigen

by abnormal immunoglobulin G (IgG).

ANS: C

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.
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REF: Page 1063

18. Sickle cell disease (SCD) is what type of inherited disorder?

a. Autosomal dominant
c. X-linked dominant
b. Autosomal recessive
d. X-linked recessive
ANS: B

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

REF: Page 1063

19. What is the reason most children diagnosed with sickle cell anemia are not candidates for

either bone marrow or stem cell transplants?

a. Well-matched stem cell donors are difficult to find.
b. The child is usually too weak to survive the procedure.
c. The childs immune system will not appropriately respond to the antirejection

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.
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REF: Page 1063

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.
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REF: Page 1066

21. What is the chance with each pregnancy that a child born to two parents with the sickle trait

will have sickle cell disease (SCD)?

a. 20%
b. 25%

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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REF: Pages 1068-1069

22. Which type of anemia occurs as a result of thalassemia?

a. Microcytic, hypochromic
c. Macrocytic, hyperchromic
b. Microcytic, normochromic
d. Macrocytic, normochromic
ANS: A

The only option that identifies the anemic manifestation of thalassemia is microcytichypochromic hemolytic anemia.
PTS: 1

REF: Page 1069

23. What is the fundamental defect that results in beta-thalassemia major?

a. The spleen prematurely destroys the precipitate-carrying cells.
b. A severe uncoupling of - and -chain synthesis occurs.
c. All four beta-forming genes are defective.
d. Hemoglobin H (HbH) develops when three genes are defective.
ANS: B

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

REF: Page 1069

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.
PTS: 1

REF: Page 1069

25. Hemophilia B is caused by a deficiency of which clotting factor?

a. V
c. IX
b. VIII
d. X
ANS: C

Only factor IX deficiency causes hemophilia B (Christmas disease).

PTS: 1

REF: Page 1071

26. Hemophilia A is considered to be what type of inherited disorder?

a. Autosomal dominant
c. X-linked recessive
b. Autosomal recessive
d. X-linked dominant
ANS: C

Hemophilia A (classic hemophilia) is inherited as an X-linked recessive disorder. This

selection is the only option that correctly identifies how this disorder is inherited.
PTS: 1

REF: Page 1071

27. Which disease is an autosomal dominant inherited hemorrhagic disease?

a. Hemophilia A
c. Christmas disease
b. von Willebrand disease
d. Hemophilia B
ANS: B

The bleeding disorder, von Willebrand disease, results only from an inherited autosomal
dominant trait.
PTS: 1

REF: Page 1071

28. Idiopathic thrombocytopenic purpura (ITP) is an autoimmune process involving antibodies

attacking which type of cells?

a. Neutrophils
b. Eosinophils

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

REF: Page 1073

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

REF: Page 1076

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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REF: Pages 1055-1056

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.
PTS: 1

REF: Page 1071

32. Which type of hemophilia affects only men?

a. Hemophilia A
c. Hemophilia C
b. Hemophilia B
d. von Willebrand disease
ANS: A

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.
PTS: 1

REF: Page 1071

33. Which hemophilia occurs equally in both men and women?

a. Hemophilia A
c. Hemophilia C
b. Hemophilia B
d. von Willebrand disease
ANS: C

Of the options available, only hemophilia C (factor XI deficiency) occurs as an autosomal

recessive disease while occurring equally in men and women.
PTS: 1

REF: Page 1071

34. During childhood, when is dietary iron deficiency commonly diagnosed?

a. Between 2 months and 1 year
c. Between 12 months and 3 years
b. Between 6 months and 2 years
d. Between 18 months and 4 years
ANS: B

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

REF: Page 1059

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

Hyperdiploidy (increased number of chromosome copies) is associated with a good prognosis.

The other options are incorrect regarding the significance of hyperdiploidy.
PTS: 1

REF: Page 1075

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

Crises can be prevented by avoiding fever, infection, acidosis, dehydration, constricting

clothes, and exposure to cold.
PTS: 1

REF: Page 1068

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
PTS: 1
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
PTS: 1
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.