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Exam Code : ONCC-CPHON
Exam Name : ONCC Certified Pediatric Hematology Oncology Nurse
Vendor Name :
"Nursing"

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Question: 1

A child with a suspected hematologic disorder is found to have a chromosomal translocation involving the BCR and ABL genes. This translocation is most commonly associated with:

1. Acute lymphoblastic leukemia (ALL)

2. Acute myeloid leukemia (AML)

3. Chronic myeloid leukemia (CML)

4. Burkitt's lymphoma
   
   Answer: C

Explanation: A chromosomal translocation involving the BCR (breakpoint cluster region) and ABL (Abelson) genes is most commonly associated with chronic myeloid leukemia (CML). This translocation, known as the Philadelphia chromosome (Ph chromosome), results in the fusion of the BCR and ABL genes. The fusion protein produced as a result of this translocation, called BCR-ABL, has oncogenic properties and drives the development of CML. Acute lymphoblastic leukemia (ALL) may also involve chromosomal translocations, but the BCR-ABL translocation is more characteristic of CML. Acute myeloid leukemia (AML) and Burkitt's lymphoma are associated with other genetic alterations.

Question: 2

A 10-year-old child with sickle cell disease is scheduled for a blood transfusion. The nurse should prioritize which action during the transfusion?

1. Assessing the child's vital signs every 15 minutes.

2. Administering premedication to prevent transfusion reactions.

3. Monitoring the child for signs of fluid overload.

Answer: A

Explanation: During a blood transfusion, the nurse should prioritize assessing the child's vital signs every 15 minutes. This is important to monitor for any adverse reactions or complications during the transfusion. Vital signs, including blood pressure, heart rate, respiratory rate, and temperature, can provide valuable information about the child's hemodynamic status and the body's response to the transfusion. While administering premedication to prevent transfusion reactions and ensuring compatibility between the donor and recipient blood types are also important steps in the transfusion process, monitoring the child's vital signs takes precedence to identify and address any immediate issues.

Question: 3

Which genetic abnormality is commonly associated with neuroblastoma?

1. MYCN amplification.

2. BCR-ABL fusion gene.

3. JAK2 mutation.

4. FLT3 mutation.
   
   Answer: A

Explanation: MYCN amplification is commonly associated with neuroblastoma, a pediatric solid tumor arising from neural crest cells. MYCN is a proto-oncogene that plays a role in cell growth and proliferation. Amplification of the MYCN gene is associated with aggressive disease and poor prognosis in neuroblastoma. BCR-ABL fusion gene is associated with chronic myelogenous leukemia (CML), JAK2 mutation is associated with certain myeloproliferative neoplasms, and FLT3 mutation is associated with

Question: 4

Which of the following is an example of a targeted therapy used in the treatment of pediatric hematologic disorders?

1. Chemotherapy drugs (e.g., vincristine)

2. Monoclonal antibody therapy (e.g., rituximab)

3. Radiation therapy (e.g., proton beam radiation)

4. Hematopoietic stem cell transplantation
   
   Answer: B

Explanation: Monoclonal antibody therapy, such as rituximab, is an example of a targeted therapy used in the treatment of pediatric hematologic disorders. Targeted therapies are designed to selectively target specific molecules or pathways involved in the growth and survival of cancer cells. Monoclonal antibodies are laboratory-produced molecules that can recognize and bind to specific cancer cells, helping to destroy them or interfere with their growth. Chemotherapy drugs, radiation therapy, and hematopoietic stem cell transplantation are not considered targeted therapies, as they have broader effects on a range of cells or processes in the body.

Question: 5

Which of the following laboratory values would the nurse expect to be elevated in a child with polycythemia?

1. Hemoglobin

2. Platelet count

3. White blood cell count

4. Mean corpuscular volume (MCV)

Answer: A

Explanation: In a child withpolycythemia, the nurse would expect the hemoglobin level to be elevated. Polycythemia is a condition characterized by an increased number of red blood cells. Hemoglobin is the protein in red blood cells that carries oxygen to the tissues, and an elevated hemoglobin level is indicative of an increased red blood cell mass. The platelet count, white blood cell count, and mean corpuscular volume (MCV) may or may not be affected in polycythemia and would not necessarily be elevated.

Question: 6

Which laboratory finding is indicative of disseminated intravascular coagulation (DIC)?

1. Prolonged prothrombin time (PT) and activated partial thromboplastin time (aPTT).

2. Elevated D-dimer level.

3. Decreased platelet count.

4. Increased fibrinogen level.
   
   Answer: B

Explanation: Elevated D-dimer level is indicative of disseminated intravascular coagulation (DIC). DIC is a complex disorder characterized by widespread activation of the coagulation cascade, leading to the formation of fibrin clots throughout the vasculature. The breakdown of these clots results in the release of D-dimer, a product of fibrin degradation. Prolonged prothrombin time (PT) and activated partial thromboplastin time (aPTT) can also be seen in DIC due to consumption of coagulation factors. Decreased platelet count and decreased fibrogen level are also common findings in DIC due to platelet and coagulation factor consumption. However, the most specific laboratory finding for DIC is

an elevated D-dimer level.

Question: 7

A 5-year-old child with a diagnosis of acute lymphoblastic leukemia (ALL) is receiving chemotherapy. The nurse should monitor the child for which potential side effect of chemotherapy?

1. Cardiotoxicity

2. Nephrotoxicity

3. Ototoxicity

4. Myelosuppression
   
   Answer: D

Explanation: Myelosuppression, or bone marrow suppression, is a common side effect of chemotherapy. It refers to a decrease in the production of blood cells, including red blood cells, white blood cells, and platelets. In the case of a child with acute lymphoblastic leukemia (ALL), the chemotherapy drugs used can affect the bone marrow and lead to myelosuppression. This can result in anemia, increased risk of infection, and bleeding tendencies. Therefore, the nurse should closely monitor the child for signs and symptoms of myelosuppression, such as fatigue, pallor, fever, and easy bruising or bleeding.

Question: 8

A child recently diagnosed with leukemia is undergoing cytogenetic testing. The nurse understands that cytogenetic testing is used to:

1. Determine the stage and grade of the leukemia.

2. Assess the child's response to chemotherapy.

3. Identify specific genetic abnormalities or mutations.

4. Measure the child's blood cell counts and levels.

Answer: C

Explanation: Cytogenetic testing is used to identify specific genetic abnormalities or mutations in a child with leukemia. It involves analyzing the chromosomes and DNA of the cancer cells to detect any genetic changes that may be associated with the development and progression of leukemia. By identifying these abnormalities, healthcare providers can better understand the child's disease and tailor the treatment approach accordingly. Cytogenetic testing does not determine the stage and grade of leukemia, assess response to chemotherapy, or measure blood cell counts and levels, as these are assessed through other diagnostic and monitoring methods.

Question: 9

A child with a history of Wilms tumor is being evaluated for signs of tumor recurrence. Which imaging modality is commonly used for surveillance in this condition?

1. Magnetic resonance imaging (MRI).

2. Positron emission tomography (PET).

3. Computed tomography (CT).

4. Ultrasonography.
   
   Answer: C

Explanation: Computed tomography (CT) is commonly used for surveillance in patients with a history of Wilms tumor. CT scans can provide detailed images of the abdomen and chest, allowing for the detection of tumor recurrence or metastasis. Magnetic resonance imaging (MRI) can also be used in certain cases, especially if there are concerns about radiation exposure. Positron emission tomography (PET) scans can be helpful in detecting metabolic activity associated with tumor recurrence or metastasis. Ultrasonography may be used

in specific situations but is generally not the primary imaging modality for surveillance in Wilms tumor.

Question: 10

Which of the following is a characteristic feature of Hodgkin's lymphoma?

1. Presence of Reed-Sternberg cells

2. Involvement of lymph nodes only

3. B-cell origin

4. Primarily affects adults
   
   Answer: A

Explanation: A characteristic feature of Hodgkin's lymphoma is the presence of Reed-Sternberg cells. Reed-Sternberg cells are large, abnormal cells that are found in the lymph nodes of individuals with Hodgkin's lymphoma. These cells are derived from abnormal B-cells and play a role in the pathogenesis of the disease. While Hodgkin's lymphoma often involves lymph nodes, it can also affect other organs and tissues. It can occur in both children and adults, although it has a bimodal age distribution with peaks in young adulthood and late adulthood.

Question: 11

A child with a suspected hematologic disorder presents with petechiae, purpura, and prolonged bleeding after minor trauma. The nurse recognizes these findings as suggestive of:

1. Thrombocytopenia

2. Leukemia

3. Hemophilia

4. Hemolytic anemia

Answer: A

Explanation: Petechiae, purpura, and prolonged bleeding after minor trauma are suggestive of thrombocytopenia, which is a condition characterized by a low platelet count. Platelets are essential for blood clotting, and a decrease in their number can lead to easy bruising, petechiae (small red or purple spots on the skin), and purpura (larger areas of purple discoloration). Thrombocytopenia can be caused by various factors, including immune-mediated destruction of platelets, decreased production of platelets, or increased consumption of platelets. Leukemia, hemophilia, and hemolytic anemia may also present with bleeding manifestations, but the specific findings described in this question are more indicative of thrombocytopenia.

Question: 12

A child with a suspected hematologic disorder is undergoing a bone marrow biopsy. The nurse should position the child in which position for the procedure?

1. Supine with the head elevated

2. Lateral recumbent position

3. Prone position

4. Trendelenburg position
   
   Answer: C

Explanation: The child should be positioned in the prone position for a bone marrow biopsy. In this position, the child lies face down on the examination table with the knees flexed and the hips externally rotated. This position allows for easier access to the posterior iliac crest, which is the most common site for bone marrow aspiration and biopsy. It also helps to minimize movement during the procedure and provides stability for the healthcare provider performing the

biopsy.

Question: 13

A child with sickle cell disease is admitted with a vaso-occlusive crisis. Which nursing intervention is essential for managing pain in this patient?

1. Administering intravenous fluids.

2. Providing oxygen therapy.

3. Applying warm compresses to affected areas.

4. Administering analgesics on a regular schedule.
   
   Answer: D

Explanation: Administering analgesics on a regular schedule is an essential nursing intervention for managing pain in a child with sickle cell disease experiencing a vaso-occlusive crisis. Pain management is a critical aspect of care during a vaso-occlusive crisis, as it helps to alleviate discomfort and improve patient outcomes. Intravenous fluids may be administered to maintain hydration and prevent further sickling of red blood cells, but they alone are not sufficient for pain management. Oxygen therapy may be used in certain situations, such as if the patient is experiencing hypoxia, but it is not the primary intervention for managing pain. Applying warm compresses to affected areas may provide some relief, but it is not the mainstay of pain management in a vaso-occlusive crisis.

Question: 14

A child with newly diagnosed acute lymphoblastic leukemia (ALL) is scheduled to undergo lumbar puncture for cerebrospinal fluid (CSF) analysis. Which nursing intervention should be implemented to minimize the risk of complications?

1. Administer pre-procedure sedation.

2. Position the child in a side-lying position with knees flexed.

3. Apply a warm compress to the lumbar puncture site.

4. Encourage the child to cough during the procedure.
   
   Answer: B

Explanation: When performing a lumbar puncture for CSF analysis in a child, positioning the child in a side-lying position with knees flexed is important to facilitate proper alignment of the spine and maximize the space between the vertebrae. This position helps to minimize the risk of complications, such as a post-procedure headache. Administering pre-procedure sedation may be considered in some cases to help reduce anxiety, but it is not the primary intervention to minimize complications. Applying a warm compress to the lumbar puncture site is not necessary and does not contribute to minimizing complications. Encouraging the child to cough during the procedure is not appropriate and does not have any benefit in reducing complications.

Question: 15

A child with a diagnosis of osteosarcoma is scheduled to undergo limb salvage surgery. Which nursing intervention should be prioritized in the immediate postoperative period?

1. Administer intravenous opioids for pain management.

2. Monitor neurovascular status of the affected limb.

3. Encourage active range of motion exercises.

4. Apply cold compresses to the surgical site.
   
   Answer: B

Explanation: In the immediate postoperative period following limb salvage surgery for osteosarcoma, monitoring the neurovascular status of the affected

limb is a priority nursing intervention. This involves assessing the circulation, sensation, and movement of the limb to ensure adequate perfusion and nerve function. Any changes in neurovascular status should be promptly reported to the healthcare team. Administering intravenous opioids for pain management is important, but it is not the highest priority intervention in this situation. Encouraging active range of motion exercises and applying cold compresses to the surgical site may be implemented later in the recovery process but are not immediate postoperative priorities.

Question: 16

A 5-year-old child presents with recurrent infections, easy bruising, and bleeding gums. Laboratory evaluation reveals a prolonged bleeding time and decreased platelet count. Which hematologic disorder should the nurse suspect?

1. Hemophilia A.

2. Idiopathic thrombocytopenic purpura (ITP).

3. Von Willebrand disease.

4. Aplastic anemia.
   
   Answer: B

Explanation: The clinical presentation of recurrent infections, easy bruising, and bleeding gums, along with a decreased platelet count and prolonged bleeding time, is suggestive of idiopathic thrombocytopenic purpura (ITP). ITP is an autoimmune disorder characterized by the destruction of platelets, leading to a decreased platelet count and an increased risk of bleeding. Hemophilia A is a genetic disorder characterized by deficient or defective factor VIII, which typically presents with spontaneous or excessive bleeding into joints and muscles. Von Willebrand disease is a bleeding disorder due to a deficiency or dysfunction of von Willebrand factor, leading to impaired platelet adhesion and aggregation. Aplastic anemia is a bone marrow failure disorder characterized by pancytopenia (decreased red blood cells, white blood cells, and platelets).

Given the clinical presentation and laboratory findings, the most likely diagnosis is ITP.

Question: 17

Which laboratory value is typically decreased in patients with iron-deficiency anemia?

1. Hemoglobin.

2. Mean corpuscular volume (MCV).

3. Serum ferritin.

4. Total iron-binding capacity (TIBC).
   
   Answer: A

Explanation: Iron-deficiency anemia is characterized by a decrease in the hemoglobin level. Hemoglobin is the protein in red blood cells that carries oxygen throughout the body. In iron-deficiency anemia, there is an insufficient amount of iron available to produce an adequate amount of hemoglobin, resulting in a decreased hemoglobin level. Mean corpuscular volume (MCV) measures the size of red blood cells and is typically decreased in iron- deficiency anemia. Serum ferritin is a marker of iron stores and is typically decreased in iron-deficiency anemia. Total iron-binding capacity (TIBC) measures the amount of transferrin available to bind iron and is typically increased in iron-deficiency anemia.

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