Deck 21: Alterations of Hematologic Function in Children

Question 1

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, resulting in a(n):

A) vaso-occlusive crisis.
B) sequestration crisis.
C) aplastic crisis.
D) hyperhemolytic crisis.

Answer 1

aplastic crisis.

Question 2

Iron deficiency anemia:

A) is most common between the ages of 2 and 4 years.
B) is related to gender and race.
C) may be related to socioeconomic factors.
D) is rare among teenagers.

Answer 2

may be related to socioeconomic factors.

Question 3

The type of sickle cell crisis seen only in young children is:

A) hyperhemolytic.
B) vaso-occlusive.
C) aplastic.
D) sequestration.

Answer 3

sequestration.

Question 4

In the United States, sickle cell disease most commonly occurs in:

A) Asians.
B) African Americans.
C) Hispanics.
D) Caucasians.

Question 5

A 10-year-old male is diagnosed with sickle cell anemia. He most likely inherited it from:

A) his mother.
B) his father.
C) both his mother and father.
D) none of the above; sickle cell disease is not clearly an inherited disease.

Question 6

The alpha and beta thalassemias are inherited in an _____ fashion.

A) autosomal recessive
B) autosomal dominant
C) X-linked recessive
D) X-linked dominant

Question 7

Sickle cell disease is characterized by the presence of Hb S. 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

Question 8

A 12-year-old male is diagnosed with sickle cell anemia. A blood smear reveals severely sickled cells. The sickled cells will be removed from circulation mostly by the:

A) liver.
B) pancreas.
C) kidney.
D) spleen.

Question 9

A 5-year-old female dies from sickle cell disease. The most likely cause of death is:

A) decreased hemoglobin.
B) infection.
C) an obstructive crisis.
D) a hyperhemolytic crisis.

Question 10

A 16-month-old female presents with tachycardia, pallor, anorexia, and systolic murmur. Hemoglobin determination indicates a content below _____ g/dl.

A) 2
B) 5
C) 10
D) 14

Question 11

Hydrops fetalis may result from which type of thalassemia?

A) Beta minor
B) Beta major
C) Alpha minor
D) Alpha major

Question 12

The erythrocytes of a person with sickle cell anemia may become sickled if:

A) oxygen tension is low.
B) pH is increased.
C) plasma osmolality is decreased.
D) fever develops.

Question 13

A 38-year-old female gives birth to a stillborn fetus. Autopsy reveals cardiomegaly, hepatomegaly, edema, and ascites. The most likely cause is:

A) alpha-thalassemia minor.
B) alpha-thalassemia major.
C) hemoglobin H disease.
D) alpha-trait.

Question 14

A 25-year-old female has a child that is diagnosed with sickle cell anemia. She does not have the disease. Which of the following characterizes the genetic makeup of the parents with regard to this trait?

A) HbS and HbS
B) HbS and HbA
C) HbS and HbC
D) HbA and HbC

Question 15

A 22-year-old female gives birth at 20 weeks' gestation to a stillborn fetus. Physical exam reveals gross edema of the entire body and anemia. This condition is referred to as:

A) hyperbilirubinemia.
B) hydrops fetalis.
C) erythroblastosis fetalis.
D) ascites.

Question 16

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.

Question 17

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 cow's milk. Considering this information, the nurse decides that the child probably has:

A) pernicious anemia.
B) iron deficiency anemia.
C) aplastic anemia.
D) hemolytic anemia.

Question 18

A major symptom of vaso-occlusive crisis in sickle cell anemia is:

A) peripheral edema.
B) pain.
C) petechiae.
D) an enlarged spleen.

Question 19

Maternal-fetal blood incompatibility may exist in which of the following conditions?

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.

Question 20

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) a erythrocyte structural abnormality.

Question 21

The mother of a 3-year-old child speaks to the pediatrician because the child took several hours to stop bleeding following a minor scrape. Testing reveals that the child has hemophilia A resulting from a deficiency in:

A) factor IX.
B) factor XII.
C) factor XIII.
D) factor VIII.

Question 22

Which condition increases the risk of developing malignant lymphoma?

A) Sickle cell disease
B) Sickle cell trait
C) Renal failure
D) AIDS

Question 23

A 1-year-old is diagnosed with idiopathic thrombocytopenic purpura (ITP). The most serious concern is development of:

A) respiratory infection.
B) asymmetric bruising.
C) intracranial bleeding.
D) immunosuppression.

Question 24

The most common childhood cancer is:

A) acute lymphoblastic leukemia.
B) chronic myelocytic leukemia.
C) non-Hodgkin lymphoma.
D) Hodgkin lymphoma.

Question 25

A 5-year-old male is diagnosed with idiopathic thrombocytic purpura (ITP). This condition is caused by:

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.

Question 26

Hemophilia B is also known as:

A) classic hemophilia.
B) Christmas disease.
C) thalassemia.
D) von Willebrand disease.

Question 27

Which of the following clusters of symptoms would lead you to suspect a child has idiopathic 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; petechiae
D) Increased platelet count; recent-onset venous thrombosis

Question 28

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. The most likely diagnosis is:

A) idiopathic thrombocytopenic purpura.
B) leukemia.
C) thalassemia.
D) hemophilia.

Question 29

A 10-year-old male with Down syndrome undergoes chemotherapy for cancer. Following treatment, he is most susceptible to:

A) acute lymphoblastic leukemia.
B) acute myelogenous leukemia.
C) non-Hodgkin lymphoma.
D) Hodgkin lymphoma.

Question 30

A 10-year-old female is diagnosed with a malignancy in the brain and lung. DNA analysis reveals translocation at chromosome 7 and 14. The most likely diagnosis is:

A) lymphoblastic non-Hodgkin lymphoma.
B) nodular non-Hodgkin lymphoma.
C) Hodgkin lymphoma.
D) small noncleaved cell non-Hodgkin lymphoma.

Question 31

A 5-year-old male is diagnosed with leukemia. Which of the following symptoms would be expected?

A) Fatigue
B) Jaundice
C) Irritability
D) Bradycardia