Chapter 31: Alterations of Hematologic Function in Children

Question 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 D- and two J-chains rather
than two D-and two E-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.

Answer 1

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

Question 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 J-chains on the hemoglobin, rather than two E-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

Answer 2

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

Question 3

Which blood cell type is elevated at birth but decreases to adult levels during the first year
of life?
a. Monocytes 
c. Neutrophils
b. Platelets 
d. Lymphocytes

Answer 3

ANS: A
Only monocyte counts are high in the first year of life and then decrease to adult levels.
PTS: 1 REF: Page 1057

Question 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 
c. 90 to 110; 140
b. 60 to 80; 120 
d. 120 to 130; 150

Answer 4

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 REF: Page 1057

Question 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

Answer 5

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.
PTS: 1 REF: Page 1058

Question 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 mother’s 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 fetus’s immune system produces
antibodies against fetal erythrocytes, which are recognized as foreign and removed
from circulation.

Answer 6

ANS: B
HDN is an alloimmune disease in which maternal blood and fetal blood are antigenically
incompatible, causing the mother’s 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 REF: Page 1058

Question 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

Answer 7

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 mother’s immune system to produce antibodies against fetal
erythrocytes. This selection is the only option that accurately defines erythroblastosis
fetalis.
PTS: 1 REF: Page 1058

Question 8

An infant’s hemoglobin must fall below \_\_\_ g/dl before signs of pallor, tachycardia, and
systolic murmurs occur.
a. 11 
c. 7
b. 9 
d. 5

Answer 8

ANS: D
When the hemoglobin falls below 5 g/dl, pallor, tachycardia, and systolic murmurs may
occur.
PTS: 1 REF: Page 1059

Question 9

Which vitamin improves the absorption of oral iron taken to treat iron deficiency anemia
in children?
a. A 
c. C
b. B 
d. E

Answer 9

ANS: C
Of the available options, only additional vitamin C may be used to promote iron
absorption.
PTS: 1 REF: Page 1059

Question 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

Answer 10

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.
PTS: 1 REF: Page 1059

Question 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 mother’s
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.

Answer 11

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.
PTS: 1 REF: Page 1060

Question 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?
a. Spherocytosis
c. Erythroblastosis fetalis
b. Icterus gravis neonatorum d. Hydrops fetalis

Answer 12

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 REF: Page 1060

Question 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
c. Jaundice
b. Icterus neonatorum
d. Icterus gravis neonatorum

Answer 13

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.
PTS: 1 REF: Page 1060

Question 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

Answer 14

ANS: B
If antigenic incompatibility of the mother’s 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 neonate’s 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

Question 15

Glucose 6-phosphate dehydrogenase (G6PD) deficiency is what type of inherited disorder?

a. X-linked dominant
c. Autosomal dominant
b. X-linked recessive
d. Autosomal recessive

Answer 15

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

Question 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

Answer 16

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.
PTS: 1 REF: Page 1062 | Page 1065

Question 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)

Answer 17

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.
PTS: 1 REF: Page 1063

Question 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

Answer 18

ANS: B
SCD is an inherited autosomal recessive disorder that is expressed as sickle cell anemia,
sickle cell–thalassemia disease, or sickle cell–hemoglobin 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

Question 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 child’s immune system will not appropriately respond to the antirejection
medications.
d. Although effective for adults, neither procedure has been proven effective for
children

Answer 19

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 REF: Page 1063

Question 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

Answer 20

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 REF: Page 1066

Question 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%
c. 33%
b. 25%
d. 50%

Answer 21

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

Question 22

Which type of anemia occurs as a result of thalassemia?

a. Microcytic, hypochromic
c. Macrocytic, hyperchromic
b. Microcytic, normochromic d. Macrocytic, normochromi

Answer 22

ANS: A
The only option that identifies the anemic manifestation of thalassemia is
microcytic-hypochromic hemolytic anemia.
PTS: 1 REF: Page 1069

Question 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 D- and E-chain synthesis occurs.
c. All four beta-forming genes are defective.
d. Hemoglobin H (HbH) develops when three genes are defective

Answer 23

ANS: B
The fundamental defect in beta-thalassemia is the uncoupling of D- and E-chain synthesis.
E-Chain production is moderately depressed in the heterozygous form—beta-thalassemia
minor, and severely depressed in the homozygous form—beta-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

Question 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

Answer 24

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

Question 25

Hemophilia B is caused by a deficiency of which clotting factor? a. V c. IX b. VIII d. X

Answer 25

ANS: C Only factor IX deficiency causes hemophilia B (Christmas disease). PTS: 1 REF: Page 1071

Question 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 dominan

Answer 26

``` 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. ```

Question 27

Which disease is an autosomal dominant inherited hemorrhagic disease? a. Hemophilia A c. Christmas disease b. von Willebrand disease d. Hemophilia B

Answer 27

ANS: B The bleeding disorder, von Willebrand disease, results only from an inherited autosomal dominant trait. PTS: 1 REF: Page 1071

Question 28

``` Idiopathic thrombocytopenic purpura (ITP) is an autoimmune process involving antibodies attacking which type of cells? a. Neutrophils c. Platelets b. Eosinophils d. Basophils ```

Answer 28

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.

Question 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)

Answer 29

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

Question 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

Answer 30

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. PTS: 1 REF: Pages 1055-1056

Question 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 ```

Answer 31

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

Question 32

Which type of hemophilia affects only men? a. Hemophilia A c. Hemophilia C b. Hemophilia B d. von Willebrand disease

Answer 32

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

Question 33

Which hemophilia occurs equally in both men and women? a. Hemophilia A c. Hemophilia C b. Hemophilia B d. von Willebrand disease

Answer 33

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

Question 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

Answer 34

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

Question 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

Answer 35

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

Question 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

Answer 36

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

Question 37

``` atch 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. Large amounts of blood become acutely pooled in the liver and spleen. 38. Blood flow is impaired by tangled masses of rigid, sickled cells. 39. Crisis occurs in association with certain drugs or infection. 40. Compensatory erythropoiesis is compromised, thus limiting the number of erythrocytes that are replaced.

Answer 37

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.