Hematologic/Oncologic/Immunologic Disorders

Question 1

Baseline management of all neonates with ABO incompatibility includes:

a) phototherapy
b) serial monitoring of bilirubin and hemoglobin levels
c) exchange transfusion
d) simple transfusion of packed RBCs

Answer 1

b) serial monitoring of bilirubin and hemoglobin levels

Question 2

Which of the following is not associated with Rh incompatibility?

a) Mother Rh negative, baby born Rh positive
b) Mother Rh positive, baby Rh negative
c) More severe in subsequent sensitized pregnancies
d) Hemolysis may occur up to 6 weeks or more

Answer 2

b) Mother Rh positive, baby Rh negative

Question 3

Clinical jaundice of the distal extremities would be noted at a bilirubin level of:
a) 15 mg/dL

Answer 3

d) > 15 mg/dL

Question 4

Beta-chain synthesis is absent in:

a) Beta-thalassemia minor
b) Beta-thalassemia intermedia
c) Beta-thalassemia major
d) Alpha-thalassemia trait

Answer 4

c) Beta-thalassemia major

Question 5

Which of the following are most often associated with hemoglobin C?

a) growth retardation
b) hepatosplenomegaly
c) usually asymptomatic
d) frontal bossing

Answer 5

c) usually asymptomatic

Symptoms include anemia, occasional jaundice, and occasional enlargement of the spleen.

Question 6

Diagnostic findings consistent with beta-thalassemia are:

a) hemoglobin normal
b) reticulocytes normal
c) Hgb A2

Answer 6

d) hypochromia, microcytosis

Question 7

Asplenic children are at increased risk for which of the following?

a) bacterial infections
b) fungal infections
c) viral infections
d) parasites

Answer 7

a) bacterial infections

Particularly streptococcus pneumoniae.

Question 8

Which of the following is not considered preventive management for iron deficiency anemia?

a) iron fortified cereal from 6 to 12 months of age
b) iron fortified formula until 6 months of age
c) no cow’s milk until 1 year of age
d) if breastfeeding supplemental iron drops or iron fortified cereal by 4-5 months of age.

Answer 8

b) iron fortified formula until 6 months of age

Question 9

The expected clinical severity of hemoglobin sickle C disease (Hgb SC) is:

a) asymptomatic
b) marked to moderate
c) mild to moderate
d) severe

Answer 9

c) mild to moderate

Question 10

The expected hemoglobin range for sickle cell anemia is:

a) 6.5-9.5 g/dL
b) 13.5-16.5 g/dL
c) 8.5-12.5 g/dL
d) 9.5-13.5 g/dL

Answer 10

a) 6.5-9.5 g/dL

Question 11

Prophylactic penicillin should be initiated in children with sickle cell anemia by:

a) 3 years of age
b) 12 months of age
c) 2-3 months of age
d) 9 months of age

Answer 11

c) 2-3 months of age

Question 12

Hemolysis does not contribute to which of the problems associated with sickle cell disease?

a) chronic anemia
b) splenic sequestration
c) aplastic crisis
d) delayed growth

Answer 12

b) splenic sequestration

Question 13

The following blood lead level is not considered lead poisoning:

a) 15
d) >25

Answer 13

a)

Question 14

Which of the following is not a precipitating factor for hemolysis in G-6-PD deficiency?

a) drugs
b) exposure to extreme temperatures
c) ingestion of fava beans
d) infection

Answer 14

b) exposure to extreme temperatures

Question 15

What percent of factor VIII/IX is associated with severe hemophilia A and B?

a) >1
b) 1-5
c) 5-25
d) 30-50

Answer 15

a) >1

Question 16

What type of hemorrhage would be expected with severe factor VIII deficiency?

a) severe hemorrhage following moderate to severe trauma
b) gross bleeding following mild to moderate trauma
c) gynecologic hemorrhage
d) spontaneous hemarthrosis

Answer 16

d) spontaneous hemarthrosis

Question 17

Which of the following is the most common type of congenital bleeding disorder?

a) hemophilia A
b) hemophilia B
c) von Willebrand disease
d) idiopathic thrombocytopenia purpura

Answer 17

c) von Willebrand disease

Question 18

Which of the following medications should be avoided in a child with ITP?

a) decongestants
b) aspirin
c) acetaminophen
d) sulfa drugs

Answer 18

b) aspirin

Question 19

The following test is required to diagnose leukemia:

a) CBC with differential
b) bone marrow aspiration/biopsy
c) chest radiograph
d) biopsy of an enlarged lymph node

Answer 19

b) bone marrow aspiration/biopsy

Question 20

Which of the following is not included as part of the initial therapy for ALL?

a) chemotherapy
b) radiation therapy
c) bone marrow transplant
d) intrathecal chemotherapy

Answer 20

c) bone marrow transplant

Question 21

Which malignancy is associated with genitourinary anomalies?

a) acute lymphocytic leukemia
b) chronic myleogenous leukemia
c) osteosarcoma
d) Wilms tumor

Answer 21

d) Wilms tumor

Question 22

Which of the following statements are true about immunizations during treatment of childhood cancer?

a) children continue to receive immunizations as usual
b) immunizations are not given during active chemotherapy
c) only live vaccines are held during active chemotherapy
d) no family member should be immunized while the child is receiving chemotherapy

Answer 22

b) immunizations are not given during active chemotherapy

Question 23

The peak incidence of osteosarcoma is:

a) 4-7 years of age
b) 8-11 years of age
c) 12-14 years of age
d) 15-19 years of age

Answer 23

d) 15-19 years of age

Question 24

The following type of infection is not associated with hypogammaglobulinemia:

a) sinusitis
b) pneumonia
c) UTI
d) cellulitis

Answer 24

c) UTI

Question 25

The following diagnostic finding is consistent with X-linked agammaglobulinemia:

a) IgG normal
b) B cells decreased
c) T cells decreased
d) IgA normal

Answer 25

b) B cells decreased

Question 26

The following is not a characteristic feature of DiGeorge syndrome:

a) hypertelorism (wide spaced eyes)
b) cleft palate
c) cardiac defects
d) frontal bossing

Answer 26

d) frontal bossing

Question 27

The following diagnostic finding is consistent with Wiskott-Aldrich syndrome:

a) IgG normal
b) IgA decreased
c) IgM increased
d) B cells decreased

Answer 27

a) IgG normal

IgE levels are elevated; T & B lymphocyte function are abnormal.

Question 28

Management of a patient with splenectomy does not include:

a) pneumococcal vaccine at least 2 weeks prior to surgery
b) prophylactic penicillin
c) blood culture and parenteral antibiotics for febrile illnesses
d) treating fever with antipyretics only and observing for resolution

Answer 28

d) treating fever with antipyretics only and observing for resolution

Question 29

A 3 yo boy with known G6PD deficiency has acute purulent OM. Which of the following drugs should not be used for his treatment?

a) amoxicillin
b) augmentin
c) erythromycin
d) bactrim

Answer 29

d) bactrim

Sulfa drugs precipitate hemolysis in patients with G6PD.

Question 30

A 2 yo is brought in for complaints of anorexia and irritability for the past several weeks. You note that she is afebrile and appears pale. Based on the s/s which initial action is appropriate?

a) ask for a description of her diet including the specific foods she was eating prior to the onset of symptoms
b) prescribe supplemental iron therapy to be given TID
c) order lab work to assess RBC count and indices
d) refer to a pediatrician for further evaluation

Answer 30

a) ask for a description of her diet including the specific foods she was eating prior to the onset of symptoms

Asking about diet prior to illness will help determine whether this patient was receiving enough iron in her diet and can help determine a differential diagnosis.

Question 31

A 5 yo male has been lethargic and has been running a low-grade fever for about 2 weeks. PE reveals no significant findings other than pallor and lymphadenopathy. A CBC reveals a decreased hematocrit, neutropenia, and thrombocytopenia. Your next action is to:

a) prescribe a broad spectrum antibiotic and ferrous sulfate
b) instruct to use acetaminophen to treat fever
c) reassure that his s/s are indicative of a viral infection
d) refer to a pediatric hematologist/oncologist for further evaluation

Answer 31

d) refer to a pediatric hematologist/oncologist for further evaluation

An abnormality of more than one formed element of the blood (RBC, WBC, or platelets) may indicate aplastic anemia (bone marrow dysfunction) or cancer, and should be evaluated by a physician.

Question 32

Routine lab studies have revealed that a 2 yo child has a decreased level of serum ferritin. Red cell count and indices are within normal limits for age. Based on this information you may assume that the child:

a) may have stage 1 iron deficiency anemia
b) likely has stage 2 iron deficiency anemia
c) likely has stage 3 iron deficiency anemia
d) does not have any stage of anema

Answer 32

a) may have stage 1 iron deficiency anemia

In the fist stage of iron deficiency the body’s iron stores are decreased. This can be detected by a fall in serum ferritin. No RBC changes are present in the first stage of iron deficiency because there is enough iron to support RBC formation.

Question 33

You have ordered RBC indices for a 10 yo girl. results reveal a decrease in both the mean corpuscular volume (MCV) and the mean corpuscular hemoglobin (MCH). Differential diagnosis would include:

a) sickle cell anemia
b) vitamin B12 deficiency anemia
c) pernicious anemia
d) iron deficiency anemia

Answer 33

d) iron deficiency anemia

Iron deficiency anemia is the most likely diagnosis of anemia characterized by mycrocytosis (decreased MCV) and hypochromia (decreased MCH).

Question 34

When planning screening protocols it is important for the practitioner to know that iron deficiency anemia is most common in which life periods?

a) the first month of life
b) the period when the child is most sedentary
c) the preschool years
d) periods of rapid growth

Answer 34

d) periods of rapid growth

The highest frequency of iron deficiency occurs during early childhood and adolescence, the same periods when growth is most rapid.

Question 35

The PNP is teaching high school students about prevention of iron deficiency anemia. To teach prevention of the most common cause of iron deficiency in this age group, it is important to emphasize:

a) avoidance of all aspirin containing products
b) a diet high in iron rich foods
c) avoidance of ingestion of carbonated beverages
d) avoidance of high-fiber foods

Answer 35

b) a diet high in iron rich foods

Nutritional deficiency is the most common cause of iron deficiency anemia.

Question 36

You have prescribed iron supplements for a 3 yo child. When instructing the parents about how to give the iron preparation, it is important to tell them that iron:

a) is best absorbed on an empty stomach
b) is best absorbed when given with meals
c) is best absorbed when given with milk
d) should not be given near bedtime

Answer 36

a) is best absorbed on an empty stomach

About twice as much iron is absorbed on an empty stomach as at mealtime. Iron is given with meals only if gastric irritation or nausea is a problem.

Question 37

One month after prescribing iron therapy to treat iron deficiency in a child who has no other known health problems, the PNP should:

a) teach the parent or guardian about iron rich foods
b) check the child’s stools for occult blood
c) order a hemoglobin measurement
d) order a complete blood count

Answer 37

c) order a hemoglobin measurement

In a healthy child recovery from iron deficiency anemia is about 2/3 complete in 1 month. Reevaluation of hemoglobin is recommended at 1 month.

Question 38

After treating a 2 yo for iron deficiency anemia laboratory tests show that his hemoglobin level has returned to normal. Which of the following actions is appropriate?

a) discontinue iron therapy and recheck hemoglobin in 1 months
b) discontinue iron therapy and tell the child’s mother to re-initiate therapy if she notices any pallor
c) continue iron therapy until all the medicine at home is gone
d) continue iron therapy for 2-3 months

Answer 38

d) continue iron therapy for 2-3 months

After hemoglobin levels have been restored, additional iron supplements are needed to replenish the body’s iron stores.

Question 39

An infant born at 34 weeks gestation is now 10 weeks old and his mother brought him to you for a routine examination. He appears alert and well developed. His mother tells you that he takes 2 to 4 oz of formula every 2 to 4 hours around the clock. The plan for this patient should include which of the following?

a) initiation of rice cereal at bedtime
b) addition of 2 bottles of water each day
c) encouraging his mother to limit feeding to every 4-6 hours
d) prescribing ferrous sulfate to be administered three times a day

Answer 39

d) prescribing ferrous sulfate to be administered three times a day

Because preterm infants have a smaller iron endowment and greater growth requirements after birth, oral iron supplements are recommended.

Question 40

While evaluating the CBC results of a 3 yo child you note that in addition to hypochromia and microcytosis of the RBC there are many poikilocytes and target cells. Based on this finding, differential must include:

a) thalassemia major
b) iron deficiency anemia
c) pernicious anemia
d) vitamin B12 deficiency

Answer 40

a) thalassemia major

Severe hypochromia and microcytosis as well as poikilocytes and target cells are seen in thalassemia. Pernicious anemia is macrocytic.

Question 41

When lab results reveal a hypochromic, microcytic anemia in a 2 yo child, differential must include:

a) lead poisoning
b) pernicious anemia
c) hemophilia
d) folic acid deficiency

Answer 41

a) lead poisoning

Lead poisoning causes hypochromic, microcytic anemia. Pernicious anemia and folic acid deficiency are associated with macrocytosis.

Question 42

A well-developed full-term 3 wo boy has been fussy and not eating well for the past week. A CBC reveals that he is anemic. When exploring the etiology of anemia it is important to know that which of the following is not a common cause of anemia in the newborn?

a) dietary iron deficiency
b) blood loss
c) hemolysis
d) decreased RBC production

Answer 42

a) dietary iron deficiency

Iron deficiency caused by dietary deficiency is uncommon in the newborn. More common etiologies in this age are blood loss, hemolysis, and decreased RBC production.

Question 43

To establish a diagnosis of sickle cell disease which laboratory test is appropriate?

a) CBC with RBC indices
b) Sickle cell prep
c) Sickledex
d) Hemoglobin electrophoresis

Answer 43

d) Hemoglobin electrophoresis

Sickle cell prep and Sickledex are screening tests and do not differentiate between sickle cell trait and sickle cell disease. Diagnosis is dependent on hemoglobin electrophoresis.

Question 44

A 5 yo has sickle cell disease. To decrease the risk of vaso-occlusive crises, it is important to stress which of the following?

a) the need for frequent hand washing
b) The need for a diet high in iron
c) avoidance of the use of mothballs in the house
d) limitation of milk intake to one glass per day

Answer 44

a) the need for frequent hand washing

Vaso-occlusive crisis is usually associated with infection, dehydration, acidosis, or exposure to cold.

Question 45

A 5 yo has sickle cell disease. Her mother tells you that she is upset because her daughter sometimes wets the bed. A UA shows no significant findings. After review of the pathophysiology of sickle cell and vasoocclusive crisis with her, which of the following should you tell the mother?

a) to limit her fluid intake after dinner and especially at bedtime
b) to keep reminding her that most girls her age do not wet the bed
c) to wake her in the middle of the night and take her to the bathroom
d) to encourage her to drink fluids and put a waterproof covering on her bed

Answer 45

d) to encourage her to drink fluids and put a waterproof covering on her bed

Hemodilution helps prevent vaso-occlusion. Fluids should always be encouraged, and bed wetting is common in young children who drink fluids near bedtime.

Question 46

A 5 yo has sickle cell disease. Her mother has brought her to the clinic because she has had fever of 101 for the past 2 days and her appetite has been poor. PE reveals no apparent cause for the fever. Appropriate treatment includes:

a) acetaminophen for fever and reevaluating in 24 hours
b) ibuprofen for fever and reevaluating in 24 hcours
c) inpatient or outpatient antibiotic therapy
d) no treatment unless the fever is above 102

Answer 46

c) inpatient or outpatient antibiotic therapy

If no simple cause is found for fever in patients with sickle cell disease, they should be treated as inpatients with IV antibiotics or outpatients with broad spectrum antibiotics.

Question 47

To prevent complications of sickle cell disease during the ages 3 months to 5 years, daily doses of which medication should be prescribed prophylactically?

a) baby aspirin
b) acetaminophen
c) diphenhydramine
d) penicillin

Answer 47

d) penicillin

Penicillin antibiotic prophylaxis is given to the child with sickle cell disease from ages 3 months to 5 years to reduce the risk of infection and subsequent vaso-occlusive crisis.

Question 48

You are seeing a toddler for an immunization update. During the appointment you learn that the pt lives in an old building that has been under renovation for the past 2 months. Based on this information you should first assess the child for:

a) asbestosis
b) coccidiodomycosis
c) mold allergy
d) lead poisoning

Answer 48

d) lead poisoning

The main source of lead for children is dust in a house with deteriorated lead-based paint. Any house built before 1960 is suspect.