Chapter 48: Hematological or Immunological Dysfunction

Question 1

1. Which statement accurately describes anemia?
   a. Increased blood viscosity
   b. Depressed hematopoietic system
   c. Presence of abnormal hemoglobin
   d. Decreased oxygen-carrying capacity of blood

Answer 1

ANS: D
Anemia is a condition in which the number of red blood cells or hemoglobin concentration is reduced below the normal values for age. This results in blood having a decreased oxygen-carrying capacity. Increased blood viscosity is usually a function of too many cells or dehydration, not anemia. A depressed hematopoietic system or abnormal hemoglobin can contribute to anemia, but the definition depends on the decreased oxygen-carrying capacity of the blood.

Question 2

2. Several blood tests are ordered for a preschool child with severe anemia. She is crying and upset because she remembers the venipuncture done at the clinic 2 days ago. What should the nurse explain to the child?
   a. Venipuncture discomfort is very brief.
   b. Only one venipuncture will be needed.
   c. Topical application of local anaesthetic can eliminate venipuncture pain.
   d. Most blood tests on children require only a finger puncture because a small
   amount of blood is needed.

Answer 2

ANS: C
Preschool children are very concerned about both pain and loss of blood. When preparing the child for venipuncture, a topical anaesthetic (e.g., EMLA) should be used to eliminate any pain and a bandage should be applied afterward. This is a traumatic experience for preschool children because they are concerned about their bodily integrity. The nurse should not promise that only one venipuncture will be needed, in case multiple attempts are required. Both finger punctures and venipunctures are traumatic for children, so both require preparation.

Question 3

3. What is the most appropriate nursing diagnosis for a child with anemia?
   a. Activity intolerance related to generalized weakness
   b. Decreased cardiac output related to abnormal hemoglobin
   c. Risk for injury related to depressed sensorium
   d. Risk for injury related to dehydration and abnormal hemoglobin

Answer 3

ANS: A
The basic pathology in anemia is the decreased oxygen-carrying capacity of the blood. The nurse must assess the child’s activity level (response to the physiological state), and the nursing diagnosis would reflect activity intolerance. In generalize d anemia, no abnormal hemoglobin may be present—only at a level of very severe anemia is cardiac output altered. No decreased sensorium exists until profound anemia occurs. Dehydration and abnormal hemoglobin are not usually part of anemia.

Question 4

4. Why is iron-deficiency anemia common during toddlerhood?
   a. Milk is a poor source of iron.
   b. Iron cannot be stored during fetal development.
   c. Fetal iron stores are depleted by age 1 month.
   d. Dietary iron cannot be started until age 12 months.

Answer 4

ANS: A
Children between the ages of 12 and 36 months are at risk for anemia because cow’s milk is a major component of their diet and it is a poor source of iron. Iron is stored during fetal development, but the amount stored depends on maternal iron stores. Fetal iron stores are usually depleted by age 5 to 6 months. Dietary iron can be introduced by breastfeeding, iron-fortified formula, and cereals during the first 12 months of life.

Question 5

5. What information should the nurse include when teaching the mother of a 9-month-old infant about administering liquid iron preparations?
   a. They should be given with meals.
   b. They should be stopped immediately if nausea and vomiting occur.
   c. Adequate dosage will turn the stools a tarry green colour.
   d. The preparation should be allowed to mix with saliva and bathe the teeth before
   swallowing.

Answer 5

ANS: C
The nurse should prepare the mother for the anticipated change in the child’s stools. If the iron dose is adequate, the stools will be a tarry green colour. The lack of the colour change may indicate insufficient iron. The iron should be given in two divided doses between meals, when the presence of free hydrochloric acid is greatest, because iron is absorbed best in an acidic environment. Vomiting and diarrhea may occur with iron administration. If these occur, iron should be given with meals at a reduced dosage, and gradually increased as the child develops tolerance. Liquid preparations of iron stain the teeth, so they should be administered through a straw and the mouth rinsed after administration.

Question 6

6. Which age group is most at risk for iron-deficiency anemia?
   a. Term infant
   b. School-aged children
   c. Adolescent
   d. Young adult

Answer 6

ANS: C
Adolescents are most at risk because of their rapid growth rate combined with poor eating habits. Preterm infants, not term infants, are especially at risk because of their reduced fetal iron supply. Healthy school-aged children and young adults are not at high risk.

Question 7

7. Which should the nurse recommend when teaching parents how to prevent iron-deficiency anemia in a healthy term breastfed infant?
   a. Iron (ferrous sulphate) drops after age 1 month.
   b. Iron-fortified commercial formula can be used by age 4 to 6 months.
   c. Iron-fortified infant cereal can be used by age 2 months.
   d. Iron-fortified infant cereal can be used at approximately 6 months of age.

Answer 7

ANS: D
Breast milk supplies inadequate iron for growth and development after age 5 to 6 months, so supplementation is necessary after this time. Iron supplementation or the introduction of solid foods to a breastfed baby is not indicated. Introducing iron-fortified infant cereal at 2 months should not be done.

Question 8

8. What is the name of the condition in which the normal, adult hemoglobin is partly or completely replaced by abnormal hemoglobin?
   a. Aplastic anemia
   b. Sickle cell anemia
   c. Hemophilia
   d. Iron-deficiency anemia

Answer 8

ANS: B
Sickle cell anemia is one of a group of diseases collectively called hemoglobinopathies, which replace normal adult hemoglobin with abnormal hemoglobin. Aplastic anemia is caused by a lack of production of cellular elements. Hemophilia refers to a group of bleeding disorders in which there is a deficiency of one of the factors necessary for coagulation. Iron-deficiency anemia is caused by factors that decrease the supply of iron, impair its absorption, increase its consumption, or affect the synthesis of HgB but the HgB is not replaced by abnormal HgB.

Question 9

9. Which statement best describes the pathological changes of sickle cell anemia?
   a. Sickle-shaped cells carry excess oxygen.
   b. Sickle-shaped cells decrease blood viscosity.
   c. Increased red blood cell destruction occurs.
   d. Decreased red blood cell destruction occurs.

Answer 9

ANS: C
The clinical features of sickle cell anemia are primarily the result of increased red blood cell destruction and obstruction caused by the sickle-shaped red blood cells. Sickled red cells have decreased oxygen-carrying capacity and transform into the sickle shape in conditions of low oxygen tension. When the sickle cells change shape, they increase the viscosity in the area where they are involved in microcirculation.

Question 10

10. Which clinical manifestation should the nurse expect when a child with sickle cell anemia experiences an acute vaso-occlusive crisis?
    a. Circulatory collapse
    b. Cardiomegaly, systolic murmurs
    c. Hepatomegaly, intrahepatic cholestasis
    d. Painful swelling of hands and feet, painful joints

Answer 10

ANS: D
A vaso-occlusive crisis is characterized by severe pain in the area of involvement. If in the extremities, painful swelling of the hands and feet is seen; if in the abdomen, severe pain resembles that of acute surgical pain in the abdomen; and if in the head, stroke and visual disturbances occur. Circulatory collapse results from sequestration crises. Cardiomegaly, systolic murmurs, hepatomegaly, and intrahepatic cholestasis result from chronic vaso-occlusive phenomena.

Question 11

11. A school-age child is admitted in vaso-occlusive sickle cell crisis. What should the child’s care include?
    a. Correction of acidosis
    b. Adequate hydration and pain management
    c. Pain management and administration of heparin
    d. Adequate oxygenation and replacement of factor VIII

Answer 11

ANS: B
The management of crises includes adequate hydration, minimizing energy expenditures, pain management, electrolyte replacement, and blood component therapy if indicated. The acidosis will be corrected as the crisis is treated. Heparin and factor VIII are not indicated in the treatment of a vaso-occlusive sickle cell crisis. Oxygen may prevent further sickling, but it is not effective in reversing sickling because it cannot reach the clogged blood vessels.

Question 12

12. The parents of a child hospitalized with sickle cell anemia tell the nurse that they are concerned about narcotic analgesics causing addiction. What should the nurse explain about narcotic analgesics?
    a. They are often ordered but not usually needed.
    b. They rarely cause addiction because they are medically indicated.
    c. They are given as a last resort because of the threat of addiction.
    d. They are used only if other measures, such as ice packs, are ineffective.

Answer 12

ANS: B
The pain of sickle cell anemia is best treated using a multidisciplinary approach. Mild to moderate pain can be controlled by ibuprofen and acetaminophen. When narcotics are indicated, they are titrated to effect and given around the clock. Patient-controlled analgesia reinforces the patient’s role and responsibility in managing the pain and provides flexibility in dealing with it. Few if any patients who receive opioids for severe pain become behaviourally addicted to the drug. Narcotics are often used because of the severe nature of the pain of vaso-occlusive crisis. Ice is contraindicated because of its vasoconstrictive effects.

Question 13

13. Which statement best describes beta-thalassemia major (Cooley’s anemia)?
    a. All formed elements of the blood are depressed.
    b. An inadequate number of red blood cells are present.
    c. Increased incidence occurs in families of Mediterranean extraction.
    d. Increased incidence occurs in families of West African descent.

Answer 13

ANS: C
Individuals who live near the Mediterranean Sea and their descendants have the highest incidence of thalassemia, which causes an overproduction of red blood cells. Although numerous, the red cells are relatively unstable. Sickle cell disease is common in blacks of West African descent.

Question 14

14. What is the purpose of initiating chelation therapy on a child with beta-thalassemia major?
    a. Treat the disease.
    b. Eliminate excess iron.
    c. Decrease the risk of hypoxia.
    d. Manage nausea and vomiting.

Answer 14

ANS: B
A complication of the frequent blood transfusions in thalassemia is iron overload. Chelation therapy with deferoxamine (an iron-chelating agent) is given with oral supplements of vitamin C to increase iron excretion. Chelation therapy treats the adverse effects of disease management. Decreasing the risk of hypoxia and managing nausea and vomiting are not the purposes of chelation therapy.

Question 15

15. In which condition are all the formed elements of the blood simultaneously depressed?
    a. Aplastic anemia
    b. Sickle cell anemia
    c. Thalassemia major
    d. Iron deficiency anemia

Answer 15

ANS: A
Aplastic anemia refers to a condition caused by abnormal bone marrow function in which the formed elements of the blood are simultaneously depressed. Sickle cell anemia is a hemoglobinopathy in which normal adult hemoglobin is partly or completely replaced by abnormal sickle hemoglobin. Thalassemia major is a group of blood disorders characterized by deficiency in the production rate of specific hemoglobin globin chains. Iron-deficiency anemia results in a decreased amount of circulating red cells.

Question 16

16. What is one possible cause of acquired aplastic anemia in children?
    a. Drugs
    b. Injury
    c. Deficient diet
    d. Congenital defect

Answer 16

ANS: A
Drugs such as chemotherapeutic agents, anticonvulsants, and some antibiotics can cause aplastic anemia. Fanconi syndrome is a primary form of the disorder, which is congenital and not acquired after birth. Deficient diet and congenital defects are not causative agents in acquired aplastic anemia.

Question 17

17. Which statement accurately describes most cases of hemophilia?
    a. Autosomal dominant disorder causing deficiency in a factor involved in the
    blood-clotting reaction
    b. X-linked recessive inherited disorder causing deficiency of platelets and
    prolonged bleeding
    c. X-linked recessive inherited disorder in which a blood-clotting factor is deficient
    d. Y-linked recessive inherited disorder in which the red blood cells become moon
    shaped

Answer 17

ANS: C
The inheritance pattern in 80% of all of the cases of hemophilia is X-linked recessive. The two most common forms of the disorder are factor VIII deficiency, hemophilia A (known as classic hemophilia), and factor IX deficiency, hemophilia B (known as Christmas disease). The disorder involves coagulation factors, not platelets. The disorder does not involve red blood cells or the Y chromosome.

Question 18

18. Which is an acquired hemorrhagic disorder that is characterized by excessive destruction of platelets?
    a. Aplastic anemia
    b. Thalassemia major
    c. Disseminated intravascular coagulation
    d. Idiopathic thrombocytopenic purpura

Answer 18

ANS: D
Idiopathic thrombocytopenic purpura is an acquired hemorrhagic disorder characterized by the excessive destruction of platelets, discolorations caused by petechiae beneath the skin, and a normal bone marrow. Aplastic anemia refers to a bone marrow failure condition in which the formed elements of the blood are simultaneously depressed. Thalassemia major is a group of blood disorders characterized by a deficiency in the production rate of specific hemoglobin globin chains. Disseminated intravascular coagulation is characterized by diffuse fibrin deposition in the microvasculature, consumption of coagulation factors and endogenous generation of thrombin and plasma.

Question 19

19. Which sentence is most descriptive of the pathophysiology of leukemia?
    a. Increased blood viscosity occurs.
    b. Thrombocytopenia (excessive destruction of platelets) occurs.
    c. Unrestricted proliferation of immature white blood cells (WBCs) occurs.
    d. The first stage of the coagulation process is abnormally stimulated.

Answer 19

ANS: C
Leukemia is a group of malignant disorders of the bone marrow and the lymphatic system. It is defined as an unrestricted proliferation of immature WBCs in the blood-forming tissues of the body. Increased blood viscosity may occur secondary to the increased number of WBCs. Thrombocytopenia may occur secondary to the overproduction of WBCs in the bone marrow. The coagulation process is unaffected by leukemia.

Question 20

20. A boy with leukemia screams whenever he needs to be turned or moved. What is the most probable cause of his pain?
    a. Edema
    b. Bone involvement
    c. Petechial hemorrhages
    d. Changes within the muscles

Answer 20

ANS: B
The invasion of the bone marrow with leukemic cells gradually causes a weakening of the bone and a tendency toward fractures. As leukemic cells invade the periosteum, increasing pressure causes severe pain. Edema, petechial hemorrhages, and muscular changes would not cause severe pain.

Question 21

21. Which sentence is a good explanation for teaching children about white blood cells?
    a. They carry the oxygen you breathe from your lungs to parts of your body.
    b. They help keep germs from causing infections.
    c. They are small parts of cells that make bleeding stop.
    d. They are the liquid portion of your blood.

Answer 21

ANS: B
White blood cells help to keep germs from causing infections. Red blood cells carry the oxygen from the lungs to other parts of the body. The small parts of cells that make bleeding stop are platelets. The liquid portion of blood is plasma.

Question 22

22. A young boy will receive a bone marrow transplant (BMT), which is possible because one of his older siblings is a histocompatible donor. What is the term for this type of BMT?
    a. Syngeneic
    b. Allogeneic
    c. Monoclonal d. Autologous

Answer 22

ANS: B
Allogeneic transplants are from another individual. Because he and his sibling are histocompatible, the bone marrow transplantation can be done. Syngeneic marrow is from an identical twin. There is no such thing as a monoclonal bone marrow transplant. Autologous refers to the individual’s own marrow.

Question 23

23. What is often administered to prevent or control hemorrhage in a child with cancer?
    a. Nitrosoureas
    b. Platelets
    c. Whole blood
    d. Corticosteroids

Answer 23

ANS: B
Most bleeding episodes can be prevented or controlled with the administration of platelet concentrate or platelet-rich plasma. Nitrosoureas, whole blood, and corticosteroids would not prevent or control hemorrhage.

Question 24

24. A school-age child with leukemia experienced severe nausea and vomiting when receiving chemotherapy for the first time. What is the most appropriate nursing action to prevent or minimize these reactions with subsequent treatments?
    a. Encourage the child to drink large amounts of his favourite fluids.
    b. Encourage the child to take nothing by mouth (remain NPO) until nausea and
    vomiting subside.
    c. Administer an antiemetic before chemotherapy begins.
    d. Administer an antiemetic as soon as child has nausea.

Answer 24

ANS: C
The most beneficial regimen to minimize nausea and vomiting associated with chemotherapy is to administer the antiemetic before the chemotherapy is begun. The goal is to prevent anticipatory symptoms. Drinking fluids will add to the discomfort of the nausea and vomiting. Encouraging the child to remain NPO will help with this episode, but the child will still have the discomfort and be at risk for dehydration. Administering an antiemetic after the child has nausea does not prevent anticipatory nausea.

Question 25

25. Which information should the nurse include when preparing a child for possible alopecia from chemotherapy?
    a. Explain to the child that hair usually regrows in 1 year.
    b. Advise the child to expose the head to sunlight to minimize alopecia.
    c. Explain to the child that wearing a hat or scarf is preferable to wearing a wig.
    d. Explain to the child that when hair regrows it may have a slightly different colour or texture.

Answer 25

ANS: D
Alopecia is an adverse effect of certain chemotherapeutic agents. When the hair regrows, it may be of different colour or texture. The hair usually grows back within 3 to 6 months after the cessation of treatment. The exposed scalp should be protected from sunlight to avoid sunburn. Children should choose the head covering that they prefer.

Question 26

26. What is one common clinical manifestation of Hodgkin’s disease?
    a. Petechiae
    b. Bone and joint pain
    c. Painful, enlarged lymph nodes
    d. Enlarged, firm, non-tender lymph nodes

Answer 26

ANS: D
Asymptomatic, enlarged, cervical, or supraclavicular lymphadenopathy is the most common presentation of Hodgkin’s disease. Petechiae are usually associated with leukemia. Bone and joint pain are not likely in Hodgkin’s disease. The enlarged nodes are rarely painful.

Question 27

27. What is caused by a virus that primarily infects a specific subset of T lymphocytes, the CD4+ T cells?
    a. Wiskott-Aldrich syndrome
    b. Idiopathic thrombocytopenic purpura (ITP)
    c. Acquired immunodeficiency syndrome (AIDS)
    d. Severe combined immunodeficiency disease

Answer 27

ANS: C
AIDS is caused by the human immunodeficiency virus, which primarily attacks the CD4+ T cells. Wiskott-Aldrich syndrome, ITP, and severe combined immunodeficiency disease are not viral illnesses.

Question 28

28. A young child with human immunodeficiency virus (HIV) is receiving several antiretroviral drugs. What do these drugs do for the child?
    a. Cure the disease.
    b. Delay the disease’s progression.
    c. Prevent the spread of the disease.
    d. Treat Pneumocystis carinii pneumonia.

Answer 28

ANS: B
Although not a cure, these antiviral drugs can suppress viral replication, preventing further deterioration of the immune system, and delay disease progression. At this time, a cure for HIV is not possible. These drugs do not prevent the spread of the disease. Pneumocystis carinii prophylaxis is accomplished with antibiotics.

Question 29

29. Which immunization should be given with caution to children infected with human immunodeficiency virus?
    a. Influenza
    b. Varicella
    c. Pneumococcal
    d. Inactivated poliovirus

Answer 29

ANS: B
Children with HIV should be carefully evaluated before they are given live viral vaccines such as varicella, herpes zoster, measles, mumps, and rubella. The child must be immunocompetent and should not have contact with other severely immunocompromised individuals. Influenza, pneumococcal, and inactivated poliovirus are not live vaccines.

Question 30

30. The nurse is planning care for an adolescent with acquired immunodeficiency syndrome. What is the priority nursing goal?
    a. Prevent infection.
    b. Prevent secondary cancers.
    c. Restore immunological defences.
    d. Identify the source of infection.

Answer 30

ANS: A
As a result of the immunocompromise that is associated with a human immunodeficiency virus infection, the prevention of infection is paramount. Although certain precautions are justified in limiting exposure to infection, these must be balanced with the child’s normal developmental needs. Restoring immunological defences is not currently possible. Current drug therapy affects disease progression; although not a cure, it can suppress viral replication, preventing further deterioration. Case finding is not a priority nursing goal.

Question 31

31. Which is an inherited immunodeficiency disorder characterized by absence of both humoral and cell-mediated immunity?
    a. Severe combined immunodeficiency disease (SCID)
    b. Acquired immunodeficiency syndrome
    c. Wiskott-Aldrich syndrome
    d. Fanconi syndrome

Answer 31

ANS: A
SCID is a genetic disorder that results in deficits of both humoral and cellular immunity. Acquired immune deficiency is not inherited. Wiskott-Aldrich syndrome is an X-linked recessive disorder with selected deficiencies of T and B lymphocytes. Fanconi syndrome is a hereditary disorder that affects red blood cell production.

Question 32

32. Several complications can occur when a child receives a blood transfusion. What is one immediate sign or symptom of an air embolus?
    a. Chills and shaking
    b. Nausea and vomiting
    c. Irregular heart rate
    d. Sudden difficulty in breathing

Answer 32

ANS: D
Signs of air embolism are sudden difficulty breathing, sharp pain in the chest, and apprehension. Air emboli should be avoided by carefully flushing all tubing of air before connecting to the patient. Chills, shaking, nausea, and vomiting are associated with hemolytic reactions. Irregular heart rate is associated with electrolyte disturbances and hypothermia.

Question 33

33. An 8-year-old girl is receiving a blood transfusion when the nurse notes that she has developed precordial pain, dyspnea, distended neck veins, slight cyanosis, and a dry cough. What are these manifestations most suggestive of?
    a. Air emboli
    b. Allergic reaction
    c. Hemolytic reaction
    d. Circulatory overload

Answer 33

ANS: D
The signs of circulatory overload include distended neck veins, hypertension, crackles, dry cough, cyanosis, and precordial pain. Signs of air embolism are sudden difficulty breathing, sharp pain in the chest, and apprehension. Allergic reactions are manifested by urticaria, pruritus, flushing, asthmatic wheezing, and laryngeal edema. Hemolytic reactions are characterized by chills, shaking, fever, pain at the infusion site, nausea, vomiting, tightness in the chest, flank pain, red or black urine, and progressive signs of shock and renal failure.

Question 34

34. The parents of a child with cancer tell the nurse that a bone marrow transplant (BMT) may be necessary. What should the nurse recognize as important when discussing this with the family?
    a. The BMT should be done at the time of diagnosis.
    b. The parents and siblings of the child have a 25% chance of being a suitable donor.
    c. Finding a suitable donor involves matching antigens from the human leukocyte antigen (HLA) system.
    d. If the BMT fails, chemotherapy or radiotherapy must be continued.

Answer 34

ANS: C
The most successful BMTs come from suitable HLA-matched donors. The timing of a BMT depends on the disease process involved. It usually follows intensive high-dose chemotherapy and/or radiation therapy. Usually parents only share approximately 50% of the genetic material with their children. A one-in-four chance exists that two siblings will have two identical haplotypes and will be identically matched at the HLA loci. Discussing the continuation of chemotherapy or radiotherapy is not appropriate when planning the BMT; that decision will be made later.

Question 35

1. Which would the nurse expect when assessing a child who has acute chest syndrome (ACS)? Select all that apply. Express answer with small letters followed by a comma and a space—e.g., a, b, c.
   a. Hypothermia
   b. Cough
   c. Severe, unrelieved headache
   d. Seizures
   e. Tachypnea
   f. Retractions

Answer 35

ANS: B, E, F
Signs of ACS include severe chest, back, or abdominal pain; fever of 38.5°C or higher; a cough; dyspnea; tachypnea; retractions; and declining oxygen saturation. A severe, unrelieved headache and seizures are signs of a cerebral vascular accident.