Transcript Pediatric Board Review Course Pediatric Hematology/Oncology

Pediatric Board Review Course
Pediatric Hematology/Oncology
Kusum Viswanathan, MD
Chair, Department of Pediatrics
Director, Division of Pediatric Hematology/Oncology
Brookdale University Hospital and Medical Center
A newborn baby has a hemoglobin of 14 gm/dL and the
MCV is 82
.True statements regarding a newborn baby include all of
the following except:
1. This is a normal Hb and MCV for a newborn
2. Feto-maternal hemorrhage can be a cause of anemia
3. A low MCV is suggestive of chronic feto-maternal
hemorrhage or Alpha Thalassemia trait.
4. Kleihauer-Betke test looks for the presence of fetal
hemoglobin in the mother’s blood
5. The Apt test looks for the presence of adult hemoglobin
in the baby’s gastric aspirate
6 week old
6 week old term infant referred for anemia.
Hb 7.5, Retic 2 %.
Mother O+, Baby A -, Direct Coombs +
Cord blood Hb 14.2 g/dL.
Jaundice of 15mg/dL at 48 hours of life, recd
photo Rx and discharged at 5 days.
No complaints, pale, Bili 3.5, Direct 0.5.
Blood smear shows spherocytes
Most likely explanation for the anemia
1. G 6 PD deficiency
2. Hereditary spherocytosis
3. Physiologic anemia
4. ABO incompatibilty
5. Rh hemolytic disease
ARS Question 1-The treatment of choice for alloimune
neonatal thrombocytopenia is
1. random platelet
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at
4.
nd
om
3.
transfusion
IVIG
Steroids
Exchange
transfusion
Washed maternal
platelets
ra
2.
50% 50%
Auto-immune Thrombocytopenia
True statements regarding Newborn Autoimmune Thrombocytopenia include all except:
1. Occurs in babies born to mothers with ITP/Hx of ITP
2. It is because of passive transfer of antibodies (IgM)
from mother.
3. The mother can have a normal or low platelet count
4. The nadir occurs a few days after birth
5. Platelets < 50,00 have a 1% risk of ICH
Treatment: IVIG to mother, Fetal platelet counts, C sec,
US, IVGG to baby
Allo-Immune Thrombocytopenia
Allo or Iso-Immune: Normal platelet count in mother
Similar to Rh disease; PL A1 antigen/ Zw-a negative
mother.
97% of population is PL A1 positive
Sensitization early in pregnancy
Plt function defect because Anti-PL-A1 interferes
w/aggregation.
Severe bleeding more likely; first born affected
Recovery in 2-3 weeks
Mother’s washed (PLA1 neg) platelets; IVIG; Ultrasound;
Steroids
Kasabach- Merritt, TAR
15 month old female presented to the ER with h/o URI, and scattered
petechiae and ecchymoses over the body and lower extremities.
Physical exam normal, no hepatosplenomegaly. WBC-7,000, Hb
12.8 gm/dl, Plts-5,000, Normal differential- Next step
1.
2.
3.
4.
perform a bone marrow aspirate to confirm the diagnosis
Non-accidental injury; skeletal survey to rule out bony
fractures
treatment with either IVIG or anti-D
Administer platelet transfusion
Acute ITP
Usually acute onset; immune mediated; post viral
Peak 2-5 years of age,
PE –no lymphadenopathy (LN), hepatosplenomegaly.
CBC- other cell lines normal, large plts on smear
Treat if plt< 10,000 or wet ITP,
Treat- IVIG best response, 48-72 hours; blocks Fc receptors,SE
– Anti-D (WInRho)- Rh+ ,hemolysis, quick response
– Steroids good response, block phagocytosis, reduces antibodies,
SE, inexpensive, need BM
BM- Increased megakaryocytes, otherwise normal
Chronic- If >6 months, F>M, older, unpredictable
prognosis
ARS Question-2
12 year old male was seen in ER with complaints of joint pain, swelling of the
feet, abdominal pain and rash. Physical examination revealed a maculopapular rash with ecchymosis and petechiae over extremities and buttocks.
CBC showed WBC-8, Hgb-12.3 gr/dl, Plts-324. Normal Differential. PT11.0s/PTT-30s. Next step in evaluating this patient is:
75%
25%
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Checking stools for occult blood
Obtaining consent for BM
aspirate
Urgent Hem/Onc consult
Barium enema
Ch
A.
Petechiae, HSP
A 2 year old boy presents for evaluation of a chronic pruritic eruption. H/o recurrent
epistaxis, otitis media, and pneumonia P/E reveals erythematous, slightly scaling
patches on the trunk and in the antecubital and popliteal fossae. Petechiae toomost suggestive of
1.
2.
3.
4.
5.
Acrodermatitis
enteropathica
Ataxia telangiectasia
Atopic dermatitis
Langerhans cell
histiocytosis
Wiskott-Aldrich syndrome
Large platelets
Normal platelet 7-10 days
Large platelets:
– ITP
– May Hegglin (Dohle
bodies in neutrophils, Plt
function normal).
– Bernard Soulier
syndrome (AR, Plat
function disorder).
Small platelets: Wiskott
Aldrich syndrome ( X-linked,
recurrent infections,
eczematoid rash, platelet
dysfunction)
ARS-3--An 18 month old girl brought in for pallor. Normal diet and
PMH. She is alert, interactive, only pallor, normal vital signs, No
hepatosplenomegaly, lymph nodes or bruises. CBC- Normal WBC, Plt,
Hb 4.5g/dl, MCV 76, Reticulocyte count 0.3%. You are considering
TEC-Transient Erythroblastopenia of childhood. All are true except:
ns
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B.
Patients are often over a year of
age
60 % recover
MCV and Hb F are high during
recovery
Treat with red cell transfusions
The rbc ADA is normal
Pa
tie
A.
Normal smear
An 8 month old girl with a history of ventricular septal
defect and horseshoe kidney presents for a health
maintenance visit.
Her parents are concerned that she is not feeding well, appearing to
become “tired” soon after beginning to breastfeed. She is tachycardic
and appears pale. Her hemoglobin level is 3.8 g/dL. Peripheral smear
reveals macrocytic red blood cells. The bone marrow aspirate shows
normal cellularity of the marrow with markedly decreased erythroid
precursors.
Which of the following findings is most likely to be identified
during additional physical examination of this patient?
1. Multiple superficial hemangiomas
2. Cutis aplasia
3. Bifid thumbs
4. Speckled white rings in the periphery of the iris
5. Posterior parietal hair whorl
Microcytic anemia is a characteristic laboratory
abnormality of all listed diseases except
1.
2.
3.
4.
Iron deficiency
Lead poisoning
Sickle cell
disease
Thalassemia trait
Microcytic anemia
Anemia
A blood smear taken from a toddler shows
microcytic hypochromic anemia. Iron
supplementation therapy is started. When will the
reticulocyte response be at maximum?
1.
1-2 days
2.
5-7 days
3.
14-21 days
4.
3-4 weeks
5.
about 6 weeks
Iron deficiency questions
Low MCV, low MCHC, low retic, RDW can be normal,
Low Iron, Incr TIBC, Transferrin low, Ferritin low
Causes: Inadequate dietary intake
– Toddlers, too much milk, less solids, Breast fed
need iron supplements
– Blood loss: Menstrual, GI tract, Meckels, Epistaxis
D/D:
Thalassemia trait- MCV much lower in prop to anemia
Anemia of chronic disease- low Fe, low TIBC, normal
/high Ferritin.
ARS Question-4- 3 year old comes for a routine check and found to be
anemic. P/E Normal. CBC Hb 8.9, MCV 58, Iron levels are normal.
Hemoglobin electrophoresis is done and shows Beta Thalassemia trait.
All are true except:
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B.
The MCV is always low
Fetal hemoglobin is often > 2%
Hemoglobin A 2 is often > 3.5%
May respond to Iron
RDW is usually normal
Fe
A.
Beta Thalassemia Minor
Quantitative defect in
globin chains
– Reduced production of
Beta chains
Hb electrophoresis
– Hb A- 2 Alpha, 2 Beta
– Hb F- 2 Alpha, 2 Gamma
– Hb A2- 2 Alpha, 2 Delta
Excess Alpha combines
with Gamma or DeltaIncreased Hb F and A2.
Smear abnormalities
significant even with
MILD anemia.
Anemia
Low MCV, normal RDW,
normal retic
Smear shows aniso and
poikulocytosis, target
cells, microcytes,
misshapen cells,
basophilic stippling
Hb Electrophoresis:
Increased Hb A2 and/or
F.
Normal iron studies, no
response to iron
Beta Thalassemia Major
No production of Beta chains- Chromosome 11
Autosomal recessive
25 % chance with each pregnancy
Pre-natal testing for carriers
Chorionic villous sampling for diagnosis
Transfusion dependent-allows for normal development
Pen Prophylaxis, Anti oxidants
Splenectomy after age 5
Iron overload- inherent and transfusion
Need chelators
BMT is a cure
ARS- Q- 5-4 year old male with no complaints. Routine CBC showed a
Hb 10gm/dL, MCV 62, RDW 12.5 , Retic 0.2%, Normal Iron studies,
Hemoglobin electrophoresis was normal with normal Hb A2 and Hb F.
What is the most likely diagnosis?
A.
B.
C.
D.
E.
Hookworm infestation
 Thallassemia
 Thallassemia
Hereditary spherocytosis
GI bleeding
Regarding Alpha Thalassemia
All of the statements are true except:
1. It is carried on 4 alleles
2. The severe form is Hydrops fetalis
3. Hemoglobin H disease occurs when 3 alleles are
affected
4. Diagnosed by newborn screening when a “fast moving”
hemoglobin is noted.
5. Fast moving hemoglobin on newborn screening is made
up of a hemoglobin with 4 beta chains
Thalassemia- Alpha
Reduced Alpha chains
4 types- carried on 4 allelles. (xx/xx)
One absent- Silent carrier (x-/xx)
2 absent- Alpha Thal trait (xx/- - or x-/x-)
3 absent- Hb H disease (x-/- -) Has 4 excess
Beta chains)
4 absent- Hydrops fetalis (- -/- -)
NB period: Excess Gamma chains form Hb
Barts- FAST moving Hb on Newborn screening
Peripheral Smear
A previously well AfricanAmerican child visited Africa
and was given malarial
prophylaxis. He experienced
pallor, fatigue, and dark urine.
His hemoglobin level decreased
from 14.8 to 9 g/dL.
1. Hereditary
spherocytosis
2. Sickle cell disease
3. Hepatitis
4. G6PD deficiency
Children with hereditary spherocytosis have all of
the listed conditions except
1. positive Direct Coombs
2. splenomegaly, gallbladder stones
3. abnormalities in spectrin and /or
ankyrin
4. increased MCHC
5. abnormal osmotic fragility test.
A 6 year old girl who has hereditary spherocytosis presents with a 1
week history of fever.
Physical exam reveals abdominal pain, vomiting,
fatigue and pallor. Her hemoglobin is typically about
10 g/dL with a retic count of 9%, but now, her hb is 4
g/dL and the retic count is 1%. The bilirubin is 1
mg/dL. Of the following, the MOST likely cause for this
girl’s present illness is infection with
–
–
–
–
–
Coxsackie virus
Parvovirus B19
Epstein-Barr virus
Hepatitis A virus
Influenza A virus
A six-year-old previously-well boy presents following a viral infection
with a 3-day history of increasing pallor and jaundice.
1.
2.
3.
4.
5.
Hemoglobin is 6.2 gm/dl. The
peripheral blood smear is shown
here. Which of the following lab
studies would be most helpful in
determining the correct diagnosis
right away?
Osmotic fragility test
G6PD assay
Heinz body prep
Direct antiglobulin test
Serum haptoglobin level
A six year-old previously-well girl presents with a
three day history of diarrhea and increasing pallor
1.
2.
3.
4.
5.
Hb is 6.2 gm/dl, Plt 40,000. The
peripheral blood smear is shown
here. Which of the following tests
would be most helpful in
determining the correct diagnosis?
Serum LDH
Bone Marrow aspirate
Direct antiglobulin test
G6PD screening test
Serum creatinine
ARS- Question 6Newborn screening of one of your new clinic patient is reported as
follows; Hb A- present, Hb F-present, Sickle hemoglobin- present.
Repeat confirmed this. You will explain to the mother that her child
has:
Sickle cell
disease
B. Normal pattern
for Afro-Americans
C. Thalassemia trait
D. Sickle cell trait.
A.
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Question
Your state neonatal screening program reports that an
infant in your practice has a pattern of hemoglobin F and S
on newborn screening. The underlying disorder that would
most likely present with this pattern is:
1. Congenital hemolytic anemia.
2. Sickle cell anemia and sickle beta+ thalassemia.
3. Sickle cell anemia and sickle beta0 thalassemia.
4. Sickle cell anemia (hemoglobin S-C disease).
5. Sickle cell trait.
Newborn Screening Questions
You get a call from a frantic parent because she
received a letter from the State regarding her
baby’s test results on NBS.
FS- SS disease, S-B0 Thal, Sickle cell w/ HPFH.
FSA- Sickle B+ thal, Sickle cell trait
FSC- SC disease
FAS- Sickle cell trait
FAC- Hb C trait
FAE- Hb E trait
FE - Hb EE disease, E-Thal
Sickle cell – which 2 are wrong
Life span 20 days
Abnormal cell shape
abnormal adherence to
endothelium
Symptoms start by 2-4 months
of age.
Hb electrophoresis, Hb S has
to be over 60%
Penicillin daily until age 10.
Prevention of pneumococcal
infections- asplenic.
PPV (Pnu-23) age 2, 5
Meningococcal vaccine early
two doses and every 5 years.
Folic acid daily
Sickle cell questions
Vaso-occlusive crisis
New infant born in another country presenting with
swollen hands---Dactylitis
Aplastic crisis:
– low Hb, low retic, Secondary to Parvovirus B 19 infection.
Splenic sequestration crisis:
– Sudden enlargement of the spleen in SS or older SC
patient
– Rx- Transfuse
– Teach spleen palpation
– Splenectomy
Transfusions in Sickle cell
All of these have been indications for transfusion
in Sickle cell disease except:
1. Acute drop of hemoglobin
2. Aplastic crises
3. Acute Chest Syndrome
4. Leg ulcers
5. Stroke
ARS- Question 7- The mother of a 10 month old baby with SS disease
asks you about prognostic indicators. All of the following indicate
likelihood of more severe disease except:
1. High WBC
2. Associated alpha
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w
thalassemia trait
3. Low hemoglobin
4. Repeated
episodes of
dactylitis
True statements regarding Acute Chest Syndrome
include all except
1. Presents with a new infiltrate on X-ray
2. Due to infarction, infection, BM fat embolism
3. Treat with antibiotics to cover pneumococcus,
Mycoplasma, Chlamydia
4. Treat with bronchodilator, Incentive spirometry,
transfusion
5. Intensive hydration is important
TCD- Transcranial Doppler
A routine TCD on a 4
year old patient with
SS disease shows a
Cerebral blood flow
(CBF) of 210
cm/second.
What is the next
step?
STOP studies- STOP
I and II
ARS-Question 8-According to the STOP protocol all children with
abnormal TCD require enrollment in hypertransfusion protocol till
(choose one)
Repeat TCD is
normal
2. Continue indefinitely
3. the child reaches 18
years
4. MRA/MRI are
reported normal
1.
ep
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True statements about Sickle cell and
Hydroxyurea are all except
1.
2.
3.
4.
5.
FDA approved for children
Increases hemoglobin F level
Increases hemoglobin
Decreases WBC – ancillary effect
Reduces number of ACS, VOC
A healthy 5 year old boy- 2 day hx of fever, P/E normal
No hepatosplenomegaly, LN, no focus of infection. CBC WBC 3,
Neutrophils 25 %, Hb 12, Platelet 200X109/L, ANC 750. Most appropriate
step is
1. Amoxicillin for 10 days
2. G- CSF for 10 days.
3. BM aspirate
4. Refer to a hematologist
5. Repeat CBC in 1-2 weeks
Neutropenia
A 2-year-old boy has had several 10-day-long episodes
of fever, mouth ulcerations, stomatitis, and pharyngitis.
These episodes have occurred at about monthly
intervals. Absolute neutrophil counts (ANC) have been
50/mm³on day 1 of each illness, 500/mm³ on day 10, and
1,500/mm³ on day 14.
1.
2.
3.
4.
5.
Among the following, the MOST likely cause for the findings in
this patient is
chronic benign neutropenia
cyclic neutropenia
Schwachman-Diamond syndrome
severe congenital neutropenia
transient viral bone marrow suppression
PT, PTT
If the PTT is prolonged, you can do a mixing study. Mix patient plasma
with equal amount of normal plasma and repeat the test. All of the
following are true except
1.
2.
3.
4.
If the PTT corrects after a mixing study, indicates that it is because
of a deficiency in factor.
If the PTT does not correct after a mixing study, indicates that it is
because of a circulating anti-coagulant
If the PTT corrects, then do a Factor VII assay
If the PTT corrects, do a Factor VIII, IX, XI assay
A healthy 2-day-old boy born at term undergoes
circumcision.
Bleeding noted at the site 10 hours after the procedure and increased
steadily over the past 4 hours. Findings on exam are unremarkable
except for bleeding along 2 to 3 mm of the surgical site; no petechiae
or purpura.
1.
2.
3.
4.
5.
Disseminated intravascular coagulation
Factor VIII deficiency hemophilia
Immune thrombocytopenic purpura
Neonatal alloimmune thrombocytopenia
Von Willebrand disease
Hemophilia
All are true for Hemophilia except
1. One unit/kg of Factor VIII increases the level to 2%.
2. The half life of Factor VIII is 12 hours
3. Inheritance is X-linked recessive
4. Severe Hemophiliacs have a Factor level of 10 %
5. 30% develop inhibitors
Hemophilia Questions
Factor VIII deficiency (Hemophilia A)-85%
– X-linked recessive, Carriers asymptomatic
– Severe<1%, Moderate 1-5, Mild 6-30 %
– Treat Recombinant Factor VIII 1unit/kg raises factor
level by 2 %. Half life 12 hrs. Joint bleeds need100%,
muscle bleeds 50 %.
– DDAVP for mild cases.
– 30 % develop inhibitors after infusions with
concentrate (Approx 50 infusions)
Factor IX deficiency (Hemophilia B)
– X-linked recessive, less common
A patient with Hemophilia A has asked you about the possibility of his
children being affected with the disease. His partner is normal
1.
2.
3.
4.
There is a 50 % chance that his sons will have the disease.
There is a 50 % chance that his daughters will be carriers
There is a 100 % chance that his sons will have the disease
There is a 100 % chance that his daughters will be carriers
Case
13 year old girl just started her periods and
has been bleeding for the past 16 days.
She has used 14 pads a day and is tired.
Her vital signs are stable, Hb 9.5, PT, PTT
normal.
The mother had heavy periods and her 6
year old brother has nose bleeds for the
past 2 years.
ARS- Question 9She was diagnosed with Type 1 Von Willebrand’s disease.
The preferred treatment for VWD Type 1 is:
A. Factor VIII
ng
e
0%
xc
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m
ae
Pl
as
ita
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DD
AV
P
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P
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ep
E.
VI
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to
r
C.
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c
B.
replacement
FFP
Cryoprecipitate
DDAVP
Plasma exchange
The most common cause of familial
predisposition to thrombosis is
1.
2.
3.
4.
5.
Hemophilia antibodies
Protein C deficiency
Protein S deficiency
Factor V Leiden mutation
Antithrombin III deficiency
Tests performed on donated units of blood
1.
2.
3.
4.
5.
All units are tested only for hepatitis B and C
All units are tested only for human immunodeficiency virus (HIV)
All units are tested for HIV, hepatitis B &hepatitis C
all units are tested for HIV, hepatitis B, hepatitis C,
sickle cell trait, cytomegalovirus, & EBV
Only units obtained from donors who have one or
more risk factors are screened for HIV, all units are
tested only for hepatitis B and C
Transfusion
IRRADIATED BLOOD products are required for
each of these patients except:
1.Children with leukemia on chemotherapy
2. Sickle cell patients with Acute Chest Syndrome
in PICU
3. Bone marrow transplanted patients
4. Preterm baby with RDS
CANCER IN CHILDREN
Childhood Cancer Distribution
Distribution-All ages
Leukemia
Lymphoma
Brain Tumor
Soft tissue sarcoma
Germ call
Bone
Neuroblastoma
Renal
Retino
Hepato
Carcinoma
Other
A 6-year-old girl has aching in her arms, legs, and back for > 2 weeks.
She has a low grade fever. P/E shows the tip of a spleen.
Results of laboratory tests include hemoglobin, 9.4 g/dL; WBC, 5,600/mm³ with no
abnormal cells noted on smear; and platelet count, 106,000/mm³.
You suspect leukemia.
True statements about Leukemia include all of the following except:
1.
2.
3.
4.
5.
Normal to see up to 5% blasts in the peripheral smear
Diagnosis is made if there are >25 % blasts in the Bone marrow
Single most common childhood cancer
Peak age 2-5 years
More likely in patients withTrisomy 21, Ataxia-Telangiectasia, Bloom
syndrome, Kostmann’s, Monosomy 7, DBA, Fanconi anemia
ALL
(Acute Lymphoblastic leukemia)
75% of all Leukemias
Can present with
generalized bone
pain, fatigue
Bruising, nose bleeds
Unusual fevers,
infection
Lymphadenopathy,
hepatosplenomegaly
ALL Treatment
Induction: 4-6 weeks,
Consolidation /delayed Intensification:
6-12 months; rotating drugs.
Maintenance : Daily oral 6-MP, weekly MTX, Monthly
pulses of Vincristine and Steroid.
Imatinib mesylate-Tyrosine Kinase Inhibitor- Ph
Chromosome positive patients
CNS prophylaxis: Intrathecal chemo
CNS Therapy: RT + Intensive systemic chemo
Testicular disease: RT
SANCTUARY- CNS, Testis
ARS- Question 10
Acute Lymphoblastic LeukemiaL
A good prognostic feature of ALL is:
A. High WBC
B. Hyperdiploidy (>50
or
ig
ce
ll
T
by
6
io
n
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ee
ks
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iss
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h
W
BC
0%
Hy
pe
r
Chromosomes)
C. Children < 1 year
of age
D. Remission by 6
weeks
E. T cell origin
ALL- Fever
A 5 year old boy who is receiving maintenance chemotherapy for acute
lymphoblastic leukemia presents with a fever of 102.2F and chills of 1 day’s
duration. He denies having a headache, URIsymptoms, vomiting, diarrhea,
or abdominal pain. Physical examination reveals an ill-appearing child who
has tachycardia but no obvious focus of infection. His skin is flushed, and
he has delayed capillary refill. Results of laboratory evaluation include:
WBC 1 x 103/ mcl, Hb 8.5 g/dL, plt count 90 x 103/ mcl, and absolute
neutrophil count, 200/ mm3. Of the following, the MOST likely cause of his
fever is
a.
Cytomegalovirus infection
b.
Disseminated Aspergillus infection
c.
Gram-negative bacteremia
d.
Haemophilus influenzae meningitis
e.
Parvovirus B19 infection
Gleevec
Gleevec (Imatinib) is a revolutionary novel
medication for treatment of Ph + CML.
Pharmacologically it belongs to this group of
medications :
1. Methylxanthines
2. HDAC inhibitors
3. Anthracyclines
4. Tyrosine kinase inhibitors.
Hodgkin’s Lymphoma
1.
2.
3.
4.
5.
16 year old male presents with a painless swelling in the
supraclavicular region. Biopsy shows Hodgkin’s disease
Stage 2. All are true except:
Presence of “B” symptoms is worse for prognosis
” B” symptoms are fever, increasing size of mass
Staging depends on whether it is on one or both sides
of the diaphragm
Sperm banking should be recommended
Second malignancy can occur in patients who receive
combination chemo and RT
Brain Tumors- Important to
know
20% of all malignancies in children
Age 3-7 years
Most often infratentorial
Sx: Persistent vomiting, headache, gait
imbalance, diplopia, ataxia, vision loss, school
deterioration, growth deceleration
Associations with Inherited Genetic disorders:
– Neurofibromatosis, Tuberous sclerosis, VonHippel-Lindau disease, Li-Fraumeni (glioma),
Turcot syndrome
Question
5 yr old boy with
progressive vomiting,
headache, unsteady
gait and diplopia for 4
weeks. MRI shows a
contrast enhancing
tumor in the 4th
ventricle with
obstructive
hydrocephalus.
Question
Which of the following statements regarding
Medulloblastoma is not true?
1.
2.
3.
4.
5.
Most common malignant brain tumor in childhood
Often presents with increased intracranial pressure
Radiation therapy is not recommended since the tumor
is not radio-sensitive
Exclusively located in the cerebellum
Can spread to the spine
Wilms Tumor
An 18-month-old girl is
being evaluated because
her mother thinks her
abdomen seems “full.”
Physical examination
reveals an abdominal
mass. Ultrasonography
identifies a solid renal
mass. At surgery, a stage
I Wilms tumor is found.
Stage I Wilms
Chance of 4-year survival is CLOSEST to
1.
2.
3.
4.
5.
30%
45%
60%
75%
95%
ARS- Question 11Congenital anomalies associated with Wilms tumor include
all of the following except
1. Polydactyly
2. Aniridia
3. Hemihypertrophy
4. Cryptorchidism
5. Denys-Drash syndrome
Scrotal mass
A 9 year old previously healthy male presents
with an increasing mass in the scrotum.
It can be any of the following except:
1. Para-testicular Rhabdomyosarcoma
2. Testicular Teratoma
3. Torsion of testis
4. Varicocoele
Rhabdomyosarcoma
Painless non tender mass
60% under age 6
Sites: head & neck, GU, Extremities, mets lungs.
Majority sporadic, associations: B-W, Li Fraumeni, NF 1
Types:
– Embryonal 70%, better prognosis
– Alveolar 30 %, trunk, worse prognosis
Treatment: Surgery, Chemo, local control RT
1.
2.
3.
4.
5.
The mother of a 22-month-old
boy reports that he has been
fussy and tired. Findings on
physical examination confirm
the presence of a nontender
right upper quadrant mass.
Bilateral periorbital
ecchymoses also are noted.
Of the following, the MOST
likely cause for these findings
is
multicystic kidney disease
neuroblastoma
non-Hodgkin lymphoma
Hepatoblastoma
Wilms tumor
All statements are true about Neuroblastoma except:
1.
2.
3.
4.
5.
Most common extra-cranial solid tumor
Prognosis worse with N-myc oncogene
amplification and tumor diploidy
Most common cancer in the first year of life
Frequent in <4 years, 97 % cases by 10 years
Infants with localized primary tumor with
dissemination limited to skin,liver and/or bone
marrow known as Stage IVS require intensive
chemotherapy.
Case
A 16 year old male comes in because he fell in
the supermarket.
P/E shows a small painless mass on the medial
aspect of the knee.
• X ray shows a fracture and a lytic sunburst pattern.
(periosteal elevation)-OS
• Xray shows an onion peel appearance- ES
• Xray- radioluscent, sharp, rounded lesion < 2cm with
reactive sclerosis- Osteoid Osteoma
X ray and MRI
Ewing’s Sarcoma
Osteoid Osteoma
Question
During your evaluation of an infant in the nursery, you
note that the red reflex in one eye seems paler than in
the other. Of the following, the MOST appropriate next
step is to
a. Apply a cycloplegic to the infant’s eyes for
examination before discharge
b. Perform the corneal light reflex test
c. Perform the cover-uncover test
d. Re-evaluate the red reflex in 1 to 2 weeks
e. Refer the infant to a pediatric ophthalmologist
Retinoblastoma- need to know
Presentation: Leukocoria (cats eye reflex), dilated pupil,
esotropia, strabismus
Unilateral 75 % (could be hereditary/non)
– 60 % unilateral and non hereditary
– 15 % unilateral and hereditary (RB1 mutation)
Bilateral 25 %
– 25 % are bilateral and hereditary, have RB1 mutation
– Earlier age, 11mos, Can develop in each eye separately
– Higher incidence of sarcoma, melanoma, brain tumors.
A child with ALL was started on chemo.
She had a WBC 82,000, Hb 9gm, plt ct 45,000. She
develops tumor lysis syndrome. Which depicts Tumor lysis
1.
2.
3.
4.
5.
K high, P high, LDH normal, Na high
K high, P normal, LDH high, Na normal
K normal, P high, LDH high, Na high
K normal, P normal, LDH high, Na normal
K high, P high, LDH high, Na normal.
Chemotherapy-Side effects
that you need to know
Anthracyclines: Cardiac toxicity
Vincristine: foot drop, peripheral neuropathy
Cisplatinum: kidney, deafness
Methotrexate, 6MP: Liver toxicity
Bleomycin: Pulmonary fibrosis
Asparaginase: Pancreatitis, Coagulopathy
Etoposide (VP-16): Secondary AML
Cyclophosphamaide: Hemorrhagic cystitis
(MESNA as Uroprotector) and Infertility
16-year-old girl, completed therapy at age 8 for Hodgkins
disease with Involved field RT and chemo.
She now develops petechiae, purpura, lymphadenopathy and
hepatosplenomegaly.Lab include: plt 12,000,Hb 8.0 gm/dL;
and WBC 13,000/mm³. She has….
1.
2.
3.
4.
5.
AML as a second malignancy
Disseminated varicella
Drug-induced ITP
Late-onset aplastic anemia due to chemotherapy
Viral-induced ITP
You are evaluating a 9 year old child for short stature. She
was treated at 3 yrs of age for Acute lymphoblastic leukemia,
received cranial RT. Her height is < 5th percentile and she is
Tanner stage I. Most likely to have an abnormal test of
1.
2.
3.
4.
5.
Growth hormone
Estradiol
Follicle stimulating hormone
Gonadotropin releasing hormone
Thyroid stimulating hormone
ARS Question- A 16 year old boy is receiving chemo for
Rhabdomyosarcoma. He received a year of cycles of Vincristine,
Actinimycin-D and Cyclophosphamide.
Most likely endocrinologic late effect of this therapy
1. Growth hormone
deficiency
2. Hypothyroidism
3. Impotence
4. Infertility
5. Osteoporosis
GVHD ( Graft vs Host disease): All are true
except
1.
2.
3.
4.
5.
It is the reaction of the donor lymphocytes
against the host.
Acute GVHD starts within the first 100 days
and chronic is after 100 days.
Affects the skin, liver and GI tract
Irradiation of blood products does not help
Complete HLA matching prevents GVHD