Heme

Burns: Pediatric Primary Care, 6th Edition

Chapter 27: Hematologic Disorders

 

 

 

Multiple Choice

 

 

1. 1. The primary care pediatric nurse practitioner sees a 12-month-old infant who is being fed goat’s milk and a vegetarian diet. The child is pale and has a beefy-red, sore tongue and oral mucous membranes. Which tests will the nurse practitioner order to evaluate this child’s condition? 

1. a. Hemoglobin electrophoresis
2. b. RBC folate, iron, and B12 levels
3. c. Reticulocyte levels
4. d. Serum lead levels

 

ANS: B

Infants and children who are fed goat’s milk or who are on a strict vegetarian diet are at risk for folic acid and vitamin B12 deficiency. These should be evaluated, along with iron, to rule out IDA. Hemoglobin electrophoresis is used to evaluate diseases associated with altered hemoglobin, such as beta-thalassemia and sickle cell anemia, neither of which is indicated by this child’s history. Reticulocyte levels are evaluated to evaluate transient erythroblastopenia of childhood, a condition that frequently follows a viral infection. Serum lead levels are not indicated based on this history.

 

 

1. 2. A 2-year-old child who has SCA comes to the clinic with a cough and a fever of 101.5°C. The child currently takes penicillin V prophylaxis 125 mg orally twice daily. What will the primary care pediatric nurse practitioner do?

1. a. Admit the child to the hospital to evaluate for sepsis.
2. b. Give intravenous fluids and antibiotics in clinic.
3. c. Increase the penicillin V dose to 250 mg.
4. d. Order a chest radiograph to rule out pneumonia.

 

ANS: A

Fever and pulmonary symptoms are two conditions warranting referral or emergency admission to the hospital to rule out sepsis and acute chest syndrome. Increasing the dose of penicillin V or giving IV antibiotics is not indicated.

 

 

1. 3. The primary care pediatric nurse practitioner evaluates a 5-year-old child who presents with pallor and obtains labs revealing a hemoglobin of 8.5 g/dL and a hematocrit of 31%. How will the nurse practitioner manage this patient?

1. a. Prescribe elemental iron and recheck labs in 1 month.
2. b. Reassure the parent that this represents mild anemia.
3. c. Recommend a diet high in iron-rich foods.
4. d. Refer to a hematologist for further evaluation.

 

ANS: A

The child has mild to moderate iron-deficiency anemia and will need iron supplementation. The hemoglobin, hematocrit, and reticulocytes should be reevaluated in 4 weeks after initiation of treatment. The child needs iron supplementation, so reassurance alone is not indicated. It is difficult to get iron from foods, so supplementation will be needed. Children with hemoglobin levels less than 4 g/dL and some children with hemoglobin levels less than 7 g/dL must be referred.

 

 

1. 4. The primary care pediatric nurse practitioner is examining a 5-year-old child who has had recurrent fevers, bone pain, and a recent loss of weight. The physical exam reveals scattered petechiae, lymphadenopathy, and bruising. A complete blood count shows thrombocytopenia, anemia, and an elevated white cell blood count. The nurse practitioner will refer this child to a specialist for

1. a. bone marrow biopsy.
2. b. corticosteroids and IVIG.
3. c. hemoglobin electrophoresis.
4. d. immunoglobulin testing.

 

ANS: A

This child has symptoms and initial lab tests consistent with leukemia and should be referred to a pediatric hematologist-oncologist for a bone marrow biopsy for a definitive diagnosis. Corticosteroids and IVIG are given for severe ITP. Hgb electrophoresis is used to diagnose SCA. Immunoglobulins are evaluated when immune deficiency syndromes are suspected.

 

 

1. 5. The pediatric nurse practitioner provides primary care for a 30-month-old child who has sickle cell anemia who has had one dose of 23-valent pneumococcal vaccine. Which is an appropriate action for health maintenance in this child?

1. a. Administer an initial meningococcal vaccine.
2. b. Begin folic acid dietary supplementation.
3. c. Decrease the dose of penicillin V prophylaxis.
4. d. Give a second dose of 23-valent pneumococcal vaccine.

 

ANS: A

Invasive bacterial infection is the leading cause of death in young children with SCA. Meningococcal vaccine should be given initially for all children over the age of 2 years and a booster dose given every 5 years after that. Folic acid supplementation is often used for adults but not for children unless there is a documented deficiency. Penicillin V prophylaxis is started at 2 months of age, with the dose increased at age 3 years. The 23-valent pneumococcal polysaccharide second dose is given 5 years after the first.

 

 

1. 6. The primary care pediatric nurse practitioner reviews hematology reports on a child with beta-thalassemia minor and notes an Hgb level of 8 g/dL. What will the nurse practitioner do?

1. a. Evaluate serum ferritin.
2. b. Order Hgb electrophoresis.
3. c. Prescribe supplemental iron.
4. d. Refer for RBC transfusions.

 

ANS: A

Children with beta-thalassemia minor may have low hemoglobin without iron deficiency so, before prescribing iron, the PNP should measure serum iron levels or serum ferritin. Hgb electrophoresis is indicated in a child whose diagnosis is unknown to diagnose this disorder. Supplemental iron should only be ordered when there is documented iron deficiency. RBC transfusions are controversial and used only for more severe iron deficiency.

 

 

1. 7. A school-age child comes to the clinic for evaluation of excessive bruising. The primary care pediatric nurse practitioner notes a history of an upper respiratory infection 2 weeks prior. The physical exam is negative for hepatosplenomegaly and lymphadenopathy. Blood work reveals a platelet count of 60,000/mm3 with normal PT and aPTT. How will the nurse practitioner manage this child’s condition?

1. a. Admit to the hospital for IVIG therapy.
2. b. Begin a short course of corticosteroid therapy.
3. c. Refer to a pediatric hematologist.
4. d. Teach to avoid NSAIDs and contact sports.

 

ANS: D

This child has symptoms, a history, and lab work that indicate idiopathic thrombocytopenic purpura. Since platelets are greater than 20,000/mm3, management without specific therapy may be done on an outpatient basis by teaching the family to avoid things that contribute to bleeding. IVIG therapy is used for children with active, severe bleeding. Corticosteroids are given for platelet counts less than 20,000/mm3. Referral to a hematologist is necessary for more severe cases.

 

 

1. 8. A toddler who presents with anemia and reticulocytopenia has a history of a gradual decrease in energy and increase in pallor beginning after a recent viral infection. How will the primary care pediatric nurse practitioner treat this child?

1. a. Closely observe the child’s symptoms and lab values.
2. b. Consult with a pediatric hematologist.
3. c. Prescribe supplemental iron for 4 to 6 months.
4. d. Refer for transfusions to correct the anemia.

 

ANS: A

This child has symptoms and a history consistent with transient erythroblastopenia of childhood (TEC), which is usually self-limited. The PNP should monitor the child closely without treatment unless the anemia gets worse. Any of the other options may be necessary if the child’s condition worsens.

 

 

1. 9. The primary care pediatric nurse practitioner is managing care for a child diagnosed with iron-deficiency anemia who had an initial hemoglobin of 8.8 g/dL and hematocrit of 32% who has been receiving ferrous sulfate as 3 mg/kg/day of elemental iron for 4 weeks. The child’s current lab work reveals elevations in Hgb/Hct and reticulocytes with a hemoglobin of 10.5 g/dL and a hematocrit of 36%. What is the next step in management of this patient?

1. a. Continue the current dose of ferrous sulfate and recheck labs in 1 to 2 months.
2. b. Discontinue the supplemental iron and encourage an iron-enriched diet.
3. c. Increase the ferrous sulfate dose to 4 to 6 mg/kg/day of elemental iron.
4. d. Refer the child to a pediatric hematologist to further evaluate the anemia.

 

ANS: A

This child has mild to moderate anemia and is showing a good response to the current dose of iron. Ferrous sulfate should be continued for at least 2 to 3 months to normalize hemoglobin, and then continue for 2 to 4 months to replace depleted iron stores. There is no need to increase the dose, since the child is responding appropriately to the current dose. Children with hemoglobin levels less than 4 g/dL should be referred.

 

 

1. 10. The primary care pediatric nurse practitioner is performing a well child examination on a school-age child who has a history of cancer treated with cranial irradiation. What will the nurse practitioner monitor in this child?

1. a. Cardiomyopathy and arrhythmias
2. b. Leukoencephalopathy
3. c. Obesity and gonadal dysfunction
4. d. Peripheral neuropathy and hearing loss

 

ANS: B

Leukoencephalopathy is a late effect of cancer treatment associated with cranial irradiation. Cardiomyopathy and arrhythmias are related to anthracycline use. Obesity and gonadal dysfunction result from neuroendocrine effects of chemotherapeutic agents. Peripheral neuropathy and hearing loss occur after cisplatin use.

 

 

1. 11. A complete blood count on a 12-month-old infant reveals microcytic, hypochromic anemia with a hemoglobin of 9.5 g/dL. The infant has mild pallor with no hepatosplenomegaly. The primary care pediatric nurse practitioner suspects 

1. a. hereditary spherocytosis.
2. b. iron-deficiency anemia.
3. c. lead intoxication.
4. d. sickle-cell anemia.

 

ANS: B

Iron-deficiency anemia is the most common type of anemia in infants and children, accounting for approximately 90% of cases. It is characterized by decreased hemoglobin, with microcytic, hypochromic RBCs. Hereditary spherocytosis is characterized by pallor and jaundice with splenomegaly. Lead intoxication is accompanied by neurobehavioral problems. Sickle-cell anemia involves the presence of HgbS.

 

 

1. 12. The primary care pediatric nurse practitioner performs a well baby examination on a 4-month-old infant who is exclusively breastfed and whose mother plans to introduce only small amounts of fruits and vegetables in addition to breastfeeding. To ensure that the infant gets adequate amounts of iron, what will the nurse practitioner recommend? 

1. a. Elemental iron supplementation of 1 mg/kg/day until cereals are added
2. b. Elemental iron supplementation of 3 mg/kg/day for the duration of breastfeeding
3. c. Monitoring the infant’s hemoglobin and hematocrit at every well-baby checkup
4. d. Offering iron-fortified formula to ensure adequate iron intake

 

ANS: A

Infants who are exclusively breastfeeding or who receive more than half of their diet from breast milk should be given 1 mg/kg/day of supplemental iron until iron-containing foods are added to the diet. It is not necessary to monitor Hgb/Hct regularly unless the child has symptoms. Formula is not necessary for breastfeeding infants.

 

 

1. 13. The primary care pediatric nurse practitioner reviews a child’s complete blood count with differential white blood cell values and recognizes a “left shift” because of

1. a. a decreased eosinophil count.
2. b. a decreased lymphocyte count.
3. c. an elevated monocyte count.
4. d. an elevated neutrophil count.

 

ANS: D

A left shift occurs when there is an increase in the number of circulating immature neutrophils and indicates a bacterial infection or an inflammatory disorder. Eosinophils are associated with an antigen-antibody response and are elevated with exposure to allergens, inflammation of skin, or parasites. Lymphocytes are non-granulocytes that are elevated with viral infections. Monocytes are non-granulocytes and are elevated in infections and inflammation and some leukemias; elevations of non-granulocytes are referred to as a “right shift.”