Chapter 22: Alterations of Hematologic Function in Children

Huether & McCance: Understanding Pathophysiology, 6th Edition

MULTIPLE CHOICE

1. A 2-year-old male presented with growth and maturation retardation and splenomegaly. He
died shortly after arriving at the ER. Autopsy revealed thalassemia secondary to defective:
a. erythrocyte membranes.
b. iron metabolism.
c. stem cell formation.
d. hemoglobin synthesis.
ANS: D
Thalassemia would be secondary to defective hemoglobin synthesis; it is not due to
erythrocyte membrane abnormalities, defective iron metabolism, or defective stem cell
formation.

REF: p. 561

2. A 1-year-old female is diagnosed with anemia secondary to insufficient erythropoiesis. The

most likely cause is:
a.genetic factors.
b.an iron deficiency.
c.a hemoglobin abnormality.
d.an erythrocyte structural abnormality.
ANS: B
The most common cause of insufficient erythropoiesis is iron deficiency, not genetic factors,
not a hemoglobin abnormality, not an erythrocyte structural abnormality.

REF: p. 554

3. A 12-month-old toddler weighing 18 pounds is brought to the clinic because of weakness,

slow physical growth, and developmental delays. His mother reports that the only food he will
consume is cows milk. The symptoms support a diagnosis of which form of anemia?
a. Pernicious
b. Iron deficiency
c. Aplastic
d. Hemolytic
ANS: B
As many as one-third of infants with severe iron deficiency anemia have chronic intestinal
blood loss induced by exposure to a heat-labile protein in cows milk. Such exposure causes
an inflammatory gastrointestinal reaction that damages the mucosa and results in diffuse
hemorrhage. This situation does not lead to pernicious anemia, aplastic anemia, or to
hemolytic anemia.

REF: pp. 554-555

4. A 16-month-old female presents with tachycardia, pallor, anorexia, and systolic murmur. The
nurse is checking the lab results, and the hemoglobin determination indicates a level below
_____ grams per deciliter.
a. 5
b. 7
c. 10
d. 14
ANS: A
When hemoglobin levels fall below 5 grams per deciliter, pallor, anorexia, tachycardia, and
systolic murmurs may occur.

REF: pp. 555-556

5. Which mother-fetus combination is at most risk for a maternal-fetal blood incompatibility?

a. Mother is Rh-positive, and fetus is Rh-negative.
b. Mother is Rh-negative, and fetus is Rh-positive.
c. Mother has type A blood, and fetus has type O blood.
d. Mother has type AB blood, and fetus has type B blood.
ANS: B
Maternal-fetal incompatibility exists if mother and fetus differ in AB and O blood type or if
the fetus is Rh-positive and the mother is Rh-negative. Symptoms do not occur in a type O
fetus. Symptoms do not occur in type AB mothers.

REF: p. 556

6. Which of the following amino acids is present in Hb S and not present in normal Hb?
a. Valine
b. Glutamic acid
c. Proline
d. Histidine
ANS: A
Hb S is formed by a genetic mutation in which one amino acid (valine) replaces glutamic acid.
Valine has replaced glutamic acid. Hb S is formed by a genetic mutation in the amino acid
(valine), not proline, or histidine.

REF: p. 557

7. In the United States, which group of people should be assessed first for sickle cell disease?
a. Asians
b. Blacks
c. Hispanics
d. Whites
ANS: B
In the United States, sickle cell disease is most common in Blacks.

REF: p. 558
 8. A mother has a child that is diagnosed with sickle cell anemia. While the mother does not
have the disease, which characteristic of her genetic makeup is responsible for the childs
disease?
a. Hb S and Hb S
b. Hb S and Hb A
c. Hb S and Hb C
d. Hb A and Hb C
ANS: B
In this instance, the child inherits Hb S from one parent and normal hemoglobin (Hb A), not
Hb S, from both. Hb C is not inherited.

REF: p. 559, Table 22-2

9. Sickled cells will be removed from circulation mostly by the:

a. liver.
b. pancreas.
c. kidney.
d. spleen.
ANS: D
Sickled cells undergo hemolysis in the spleen or become sequestered there, causing blood
pooling and infarction of splenic vessels. The hemolysis of sickled cells does not occur mainly
in the liver, the pancreas, or the kidneys.

REF: p. 559

10. Which major symptom indicates to the nurse that patient diagnosed with sickle cell anemia is
experiencing a vasoocclusive crisis?
a. Peripheral edema
b. Pain
c. Petechiae
d. An enlarged spleen
ANS: B
Vasoocclusive crisis is extremely painful and may last for days or even weeks, with an
average duration of 4-6 days. Vasoocclusive crisis does not promote peripheral edema,
petechiae, or an enlarged spleen.

REF: p. 560

11. Which type of sickle cell crisis occurs only in young children?
a. Hyperhemolytic crisis
b. Vaso-occlusive crisis
c. Aplastic crisis
d. Sequestration crisis
ANS: D
Sequestration occurs when large amounts of blood become acutely pooled in the liver and
spleen. This type of crisis is seen only in a young child. Hyperhemolytic, vaso-occlusive, and
aplastic crises are not age specific.
 REF: p. 560

12. A 6-year-old male presents with fatigue, jaundice, and irritability. A blood smear shows the
presence of sickled cells. Erythropoiesis is compromised in this child; which crisis should the
nurse monitor the patient for?
a. Vaso-occlusive crisis
b. Sequestration crisis
c. Aplastic crisis
d. Hyperhemolytic crisis
ANS: C
Aplastic anemia is caused by diminished erythropoiesis despite an increased need for new
erythrocytes. Vaso-occlusive crisis is manifested by pain. Sequestered crisis is manifested by
enlarged spleen. Hyperhemolytic crisis is due to infection.

REF: p. 560

13. What is the most likely cause of death associated with sickle cell disease?
a. Decreased hemoglobin
b. Infection
c. An obstructive crisis
d. A hyperhemolytic crisis
ANS: B
Infection is the most common cause of death related to sickle cell disease. The most common
cause of sickle cell disease related to death is not associated with decreased hemoglobin or
with either an obstructive or hyperhemolytic crisis.

REF: p. 560

14. The alpha and beta thalassemias are inherited in an _____ fashion.
a. autosomal recessive
b. autosomal dominant
c. X-linked recessive
d. X-linked dominant
ANS: A
The alpha and beta thalassemias are inherited autosomal recessive disorders. The alpha and
beta thalassemias are not inherited autosomal dominant disorders. They are not an X-linked
disorder.

REF: p. 561

15. Autopsy on a stillborn fetus reveals cardiomegaly, hepatomegaly, edema, and ascites as well
as fulminant intrauterine congestive heart failure. Considering these findings, what is the most
likely cause of death?
a. Alpha thalassemia minor
b. Alpha thalassemia major
c. Hemoglobin H disease
d. Alpha trait
ANS: B
 Alpha thalassemia major causes hydrops fetalis and fulminant intrauterine congestive heart
failure. In addition to edema and massive ascites, the fetus has a grossly enlarged heart and
liver. These findings are not associated with alpha thalassemia minor, hemoglobin H disease,
or alpha trait, which is the mildest form of the disease.

REF: p. 562

16. If an infant has hydrops fetalis, which type of thalassemia does the nurse suspect?
a. Beta minor
b. Beta major
c. Alpha minor
d. Alpha major
ANS: D
Hydrops fetalis is due to alpha thalassemia major, not either beta form or alpha minor.

REF: p. 562

17. Testing reveals that a child has hemophilia A. This bleeding disorder results from a deficiency
in factor:
a. IX.
b. XII.
c. XIII.
d. VIII.
ANS: D
Hemophilia A is a deficiency in factor VIII. Hemophilia A is not associated with a deficiency
in factor IX, XII, or XIII.

REF: p. 563

18. By what other name is the clotting factor IX disorder, hemophilia B known as?
a. Classic hemophilia
b. Christmas disease
c. Thalassemia
d. von Willebrand disease
ANS: B
Hemophilia B is also known as Christmas disease; not classic hemophilia. Thalassemia is not
a clotting disorder. von Willebrand disease is a factor VIII disorder.

REF: p. 563

19. A 3-year-old child presents with bruising on the legs and trunk and a petechial rash. The
mother also reports frequent nosebleeds. Lab tests reveal a decreased platelet count. This
symptomology supports which medical diagnosis?
a. Immune thrombocytopenic purpura (ITP)
b. Leukemia
c. Thalassemia
d. Hemophilia
ANS: A
 A decreased platelet count is the cause of ITP. Neither leukemia, thalassemia, nor hemophilia
is related to platelets.

REF: p. 564

20. A 5-year-old male is diagnosed with immune thrombocytic purpura (ITP). What is the most
common cause of this condition?
a.Normal postnatal platelet lysis
b.Virally induced antibody destruction of platelets
c.An allergic reaction to vaccinations
d.Maternal antibodies that target platelets in the neonate
ANS: B
In approximately 70% of cases of ITP, there is an antecedent viral disease. ITP is not
associated with postnatal platelet lysis, an allergic reaction, or contact with maternal
antibodies.

REF: p. 564

21. Which of the following clusters of symptoms would lead the nurse to suspect a child has
immune thrombocytopenic purpura (ITP)?
a.Multiple infections; bruising; abnormal bone marrow aspiration
b.Lower extremity that is warm to touch, edematous, and painful
c.Spontaneous nosebleeds, bruising, and petechiae
d.Increased platelet count; recent-onset venous thrombosis
ANS: C
Nosebleeds, bruising, and petechiae are signs of a lack of clotting due to low platelets and ITP.
The symptoms associated with ITP are not associated with infections, warm extremities that
are edematous and painful, or an increased platelet count.

REF: p. 564

22. What is the most serious complication of immune thrombocytopenic purpura (ITP)?
a. Respiratory infection
b. Asymmetric bruising
c. Intracranial bleeding
d. Immunosuppression
ANS: C
Although the incidence is less than 1%, intracranial hemorrhage is the most serious
complication of ITP.

REF: p. 564

23. What is the most common form of childhood leukemia?

a. Acute lymphoblastic leukemia (ALL)
b. Chronic myelocytic leukemia (CML)
c. Acute myeloid leukemia (AML)
d. Chronic lymphocytic leukemia (CLL)
ANS: A
 Approximately 75% of leukemias in children are ALL.

REF: p. 564

24. What is a priority assessment for Hodgkin lymphoma in children?

a. Painless adenopathy in cervical nodes
b. Significant bruising
c. Weight gain
d. Petechiae
ANS: A
Painless adenopathy in the lower cervical chain, with or without fever, is the most common
symptom in children with Hodgkin lymphoma. Bruising, weight gain, or petechiae are
associated with Hodgkin lymphoma in children.

REF: p. 566

25. Which laboratory test will assist the nurse in identifying infants who are prone to develop
hemolytic disease of the newborn (HDN)?
a.Total bilirubin
b.Coombs
c.Rh antibodies
d.Platelets
ANS: B
Routine evaluation of fetuses at risk for HDN includes the Coombs test. Routine evaluation of
fetuses at risk for HDN would not include total bilirubin, Rh antibodies, or platelets.

REF: p. 557

MULTIPLE RESPONSE

1. A nurse is planning care for a child with iron deficiency anemia. Characteristics of iron
deficiency anemia include which of the following? (select all that apply)
a. It is most common between the ages of 6 months and 2 years.
b. It is related to gender and race.
c. It may be related to socioeconomic factors.
d. It is rare among teenagers.
e. It is difficult to diagnose in early stages.
ANS: A, C, E
Iron deficiency anemia is the most common nutritional disorder of infancy and childhood,
with the highest incidence occurring between 6 months and 2 years of age. Incidence is not
related to gender or race, but socioeconomic factors are important because they affect
nutrition. The symptoms of mild anemialistlessness and fatigueusually are not present or
are undetectable in infants and young children who are unable to describe these symptoms.
Therefore, parents generally do not note any change in the childs behavior or appearance
until moderate anemia has developed.

REF: pp. 554-555

2. A 5-year-old male is diagnosed with leukemia. Which of the following symptoms would the
nurse expect? (select all that apply)
a. Fatigue
b. Jaundice
c. Pallor
d. Petechiae
e. Fever
ANS: A, C, D, E
Pallor, fatigue, petechiae, purpura, bleeding, and fever generally are present. Jaundice is not.

REF: p. 564