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ONCC-CPHON
ONCC Certified Pediatric Hematology Oncology Nurse
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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)
hronic myeloid leukemia (CML) urkitt's lymphoma
wer: C
anation: A chromosomal translocation involving the BCR (breakpoint er region) and ABL (Abelson) genes is most commonly associated wi nic myeloid leukemia (CML). This translocation, known as the delphia chromosome (Ph chromosome), results in the fusion of the B
BL genes. The fusion protein produced as a result of this translocatio called BCR-ABL, has oncogenic properties and drives the development of
L. Acute lymphoblastic leukemia (ALL) may also involve chromosom locations, but the BCR-ABL translocation is more characteristic of C Acute myeloid leukemia (AML) and Burkitt's lymphoma are associated wi
genetic alterations.
stion: 2
Acute myeloid leukemia (AML)
2. C
3. B
Ans Expl
clust th
chro
Phila CR
and A n,
CM al
trans ML.
th
other
Que
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
able information about the child's hemodynamic status and the body's onse to the transfusion. While administering premedication to prevent fusion reactions and ensuring compatibility between the donor and ient blood types are also important steps in the transfusion process, toring the child's vital signs takes precedence to identify and address ediate issues.
stion: 3
ch genetic abnormality is commonly associated with neuroblastoma? YCN amplification.
CR-ABL fusion gene. AK2 mutation.
LT3 mutation. wer: 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
valu resp trans recip
moni any
imm Que Whi
1. M
2. B
3. J
4. F
Ans
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?
onoclonal antibody therapy (e.g., rituximab) adiation therapy (e.g., proton beam radiation) ematopoietic stem cell transplantation
wer: B
anation: Monoclonal antibody therapy, such as rituximab, is an examp geted therapy used in the treatment of pediatric hematologic disorders. eted therapies are designed to selectively target specific molecules or ways involved in the growth and survival of cancer cells. Monoclonal odies are laboratory-produced molecules that can recognize and bind fic cancer cells, helping to destroy them or interfere with their growth motherapy drugs, radiation therapy, and hematopoietic stem cell plantation are not considered targeted therapies, as they have broader ts on a range of cells or processes in the body.
stion: 5
Chemotherapy drugs (e.g., vincristine)
M
R
H
Ans
Expl le of
a tar Targ path
antib to
speci .
Che trans effec
Que
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
ount, and mean corpuscular volume (MCV) may or may not be affect cythemia and would not necessarily be elevated.
stion: 6
ch laboratory finding is indicative of disseminated intravascular ulation (DIC)?
rolonged prothrombin time (PT) and activated partial thromboplastin t T).
levated D-dimer level. ecreased platelet count. ncreased fibrinogen level.
wer: B
anation: Elevated D-dimer level is indicative of disseminated intravas ulation (DIC). DIC is a complex disorder characterized by widespread
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 c ed in
poly
Que
Whi coag
1. P ime
(aPT
2. E
3. D
4. I
Ans
Expl cular
coag
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?
ardiotoxicity ephrotoxicity totoxicity yelosuppression
wer: D
anation: Myelosuppression, or bone marrow suppression, is a commo of chemotherapy. It refers to a decrease in the production of blood c ding red blood cells, white blood cells, and platelets. In the case of a c cute lymphoblastic leukemia (ALL), the chemotherapy drugs used c the bone marrow and lead to myelosuppression. This can result in
mia, increased risk of infection, and bleeding tendencies. Therefore, th should closely monitor the child for signs and symptoms of osuppression, such as fatigue, pallor, fever, and easy bruising or bleed
stion: 8
C
N
O
M
Ans
Expl n side
effect ells,
inclu hild
with a an
affect
ane e
nurse
myel ing.
Que
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
's disease and tailor the treatment approach accordingly. Cytogenetic ng does not determine the stage and grade of leukemia, assess respons motherapy, or measure blood cell counts and levels, as these are assess ugh other diagnostic and monitoring methods.
stion: 9
ild with a history of Wilms tumor is being evaluated for signs of tumo rence. Which imaging modality is commonly used for surveillance in ition?
agnetic resonance imaging (MRI). ositron emission tomography (PET). omputed tomography (CT). ltrasonography.
wer: 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
testi e to
che ed
thro
Que
A ch r
recur this
cond
1. M
2. P
3. C
4. U
Ans
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?
volvement of lymph nodes only cell origin
rimarily affects adults wer: A
anation: A characteristic feature of Hodgkin's lymphoma is the presen
-Sternberg cells. Reed-Sternberg cells are large, abnormal cells that a in the lymph nodes of individuals with Hodgkin's lymphoma. These erived from abnormal B-cells and play a role in the pathogenesis of t se. While Hodgkin's lymphoma often involves lymph nodes, it can al
other organs and tissues. It can occur in both children and adults, ugh it has a bimodal age distribution with peaks in young adulthood a dulthood.
stion: 11
ild with a suspected hematologic disorder presents with petechiae, pur
Presence of Reed-Sternberg cells
In
B-
P
Ans
Expl ce of
Reed re
found cells
are d he
disea so
affect
altho nd
late a
Que
A ch pura,
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
aused by various factors, including immune-mediated destruction of lets, decreased production of platelets, or increased consumption of lets. Leukemia, hemophilia, and hemolytic anemia may also present w ding manifestations, but the specific findings described in this questio
indicative of thrombocytopenia.
stion: 12
ild with a suspected hematologic disorder is undergoing a bone marro sy. The nurse should position the child in which position for the edure?
upine with the head elevated ateral recumbent position rone position
rendelenburg position
wer: C
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 c
plate
plate ith
blee n are
more
Que
A ch w
biop proc
1. S
2. L
3. P
4. T
Ans
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?
roviding oxygen therapy.
pplying warm compresses to affected areas. dministering analgesics on a regular schedule.
wer: D
anation: Administering analgesics on a regular schedule is an essentia ng intervention for managing pain in a child with sickle cell disease riencing a vaso-occlusive crisis. Pain management is a critical aspect o uring a vaso-occlusive crisis, as it helps to alleviate discomfort and ove patient outcomes. Intravenous fluids may be administered to main ation and prevent further sickling of red blood cells, but they alone are cient for pain management. Oxygen therapy may be used in certain tions, such as if the patient is experiencing hypoxia, but it is not the ary intervention for managing pain. Applying warm compresses to aff may provide some relief, but it is not the mainstay of pain managem
o-occlusive crisis.
Administering intravenous fluids.
P
A
A
Ans
Expl l
nursi
expe f
care d
impr tain
hydr not
suffi situa
prim ected
areas ent in
a vas
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
ioning the child in a side-lying position with knees flexed is importan tate proper alignment of the spine and maximize the space between th brae. This position helps to minimize the risk of complications, such a procedure headache. Administering pre-procedure sedation may be idered in some cases to help reduce anxiety, but it is not the primary vention to minimize complications. Applying a warm compress to the ar puncture site is not necessary and does not contribute to minimizin plications. Encouraging the child to cough during the procedure is not opriate and does not have any benefit in reducing complications.
stion: 15
ild with a diagnosis of osteosarcoma is scheduled to undergo limb sal ery. Which nursing intervention should be prioritized in the immediate operative period?
dminister intravenous opioids for pain management. onitor neurovascular status of the affected limb.
Explanation: When performing a lumbar puncture for CSF analysis in a child, posit t to
facili e
verte s a
post- cons inter
lumb g
com appr
Que
A ch vage
surg post
1. A
2. M
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
stion: 16
year-old child presents with recurrent infections, easy bruising, and ding gums. Laboratory evaluation reveals a prolonged bleeding time a eased platelet count. Which hematologic disorder should the nurse sus
emophilia A.
iopathic thrombocytopenic purpura (ITP). on Willebrand disease.
plastic anemia. wer: B
anation: The clinical presentation of recurrent infections, easy bruisin leeding gums, along with a decreased platelet count and prolonged ding time, is suggestive of idiopathic thrombocytopenic purpura (ITP). autoimmune disorder characterized by the destruction of platelets, lea
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.
Que
A 5-
blee nd
decr pect?
1. H
2. Id
3. V
4. A
Ans
Expl g,
and b
blee ITP
is an ding
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?
emoglobin.
ean corpuscular volume (MCV). erum ferritin.
otal iron-binding capacity (TIBC). wer: A
anation: Iron-deficiency anemia is characterized by a decrease in the oglobin level. Hemoglobin is the protein in red blood cells that carries en throughout the body. In iron-deficiency anemia, there is an insuffi unt of iron available to produce an adequate amount of hemoglobin, ting in a decreased hemoglobin level. Mean corpuscular volume (MC ures the size of red blood cells and is typically decreased in iron- iency anemia. Serum ferritin is a marker of iron stores and is typicall eased in iron-deficiency anemia. Total iron-binding capacity (TIBC) ures the amount of transferrin available to bind iron and is typically ased in iron-deficiency anemia.
H
M
S
T
Ans Expl
hem
oxyg cient
amo
resul V)
meas
defic y
decr meas incre

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