Heme/onc Flashcards
(173 cards)
1
Q
Child (age?) with ataxia, diplopia and headaches, and head tilt. What is the most likely dx?
a. brainstem glioma
b. cerebellar astrocytoma
c. craniopharyngioma
d. ependymoma
A
B cerebellar astrocytoma
torticollis - ?cerebellar herniation
supra tentorial - focal sensory/lateralizing effect
infra tentorial - h/a nausea vomitting
2
Q
Picture given of large hemangioma over V1 to scalp and upper eyelid distribution with bluish tint. Baby presents with this facial lesion and high output CHF. Apart from thrombocytopenia what else would you expect to see on CBC or smear:
a. Neutropenia and mild anemia
b. Normal INR/PTT
c. Schistocytes
d. High Fibrinogen -
A
C schistoytes
low fibronegen
KASSELBACH MERRIT syndrome - activation of coag, with trapped consumption, and coagulopathy and abn INR
AVM within can cause CHF
MAHA
3
Q
Teen with weight loss, generalized lymphadenopathy including supraclavicular node, and palpable spleen tip. More fatigue recently. Afebrile. WBC 10, Hgb 120, Plt 150. Normal Monospot. What is the next step in evaluation:
a. BM aspirate
b. Chest Xray
c. Excise node
d. ANA
A
CXR
4
Q
Wilm’s tumour is most associated with:
a. Tuberous sclerosis
b. Fragile X
c. Angelman’s
d. Beckwith Weidemann
A
BW
also get rhabdo, adenoCa, hepatobl
5
Q
sickle cell most common neurologic sequelae
a. Silent stroke
b. Clinical Stroke
c. Seizures
A
silent stroke
6
Q
Teenage girl with SSD. Acute fever, jaundice, abdo pain. Suspect cholecystits, confirmed with U/S. After treating for one week, what do you suggest?
a. Cholecystectomy
b. Ursodiol
c. ERCP
A
a. Cholecystectomy
7
Q
A 9 month old ex-32 weeker takes 40 oz of homo milk per day. Hgb is 60, MCV 50. She is treated with Fe 4 mg/kg/day for one month. On repeat testing, her Hgb is 62, MCV is 50, and her retics are 0.01. What to do next?
a. BMA
b. verify compliance
c. Hb electrophoresis
d. Jejunal biopsy
A
verify compliance
Iron deficiency anemia, what will you find on exam:
a. Pica
4-6mg/kg/day of iron is treatment
8
Q
15 year old girl with periods for a couple of years. Epistaxis and menorrhagia. What to check for?
a. von Willebrands
b. Hemophilia C
c. Factor V Leiden mutation
A
a. von Willebrands
type 1 parital, type 2 dec fxnal, type 3 absent
9
Q
An otherwise healthy girl presents with pallor. Her iron, and ferritin levels are normal. Her bloodwork shows a Hb of 70 with an MCV of 50. What study is most likely to give you her diagnosis?
a. Bone marrow aspirate
b. Hemoglobin electrophoresis
A
electrophoresis
10
Q
18 month old male (13kg) with history of excessive milk intake presents on routine physical exam with pallor Subsequent bloodwork reveals Hb 45, MCV 56. What would be the best management?
a. Limit milk to 500 cc/day
b. Start elemental iron 60mg PO tid
c. Give PRBC transfusion 130cc
d. Change to protein hydrosylate formula
A
Limit milk to 500 cc/day
11
Q
What is the advantage of using leukoreduced white cells?
a. Decreases hemolytic reactions
b. Decreases infectious complications
c. Decreases febrile transfusion reactions
A
Decreases infectious complications
12
Q
Hb 48 in an 11 m.o. M with tachycardia, pallor, excess milk intake.
a. restrict milk intake to < 500 cc/day
b. Fe TID at 15 mg/kg
c. Transfuse 10 cc/kg blood
d. Change to protein hydrolysate formula
A
c. Transfuse 10 cc/kg blood d/t tachy
13
Q
7 year old boy who has had recent personality changes, decline in school performance and visual changes. Which is the first diagnosis to rule out?
a. Brain tumour
b. DM
c. ADHD
d. Depression
A
a. Brain tumour
14
Q
8 year old health child with supraclavicular lymph node. What to do?
a. excise
b. PPD
c. bartonella serology
d. EBV
A
a. excise
15
Q
What is a risk factor for child leukemia?
a. parent treated for leukemia
b. in utero radiation
c. maternal alcohol
d. NF type 1
A
NF1, down syndrome, SCID, AT and bloom
Environmental factors: Ionizing radiation, Drugs, Alkylating agents, Epipodophyllotoxin, Benzene exposure
16
Q
Which of the following is an indication for bone marrow biopsy in a child with ITP who has platelets of 12 000?
a. ANC <1000
b. Previous use of steroids
c. Hb <115 - can get mild anemia due to bleeding, if unexplained will need BMA
d. Fever > 39
A
ANC 1000
17
Q
16 y.o. boy undergoing treatment for non-Hodgkin’s lymphoma. Forty-eight hours after his last chemotherapy, he develops mild dysuria and hematuria. Platelet count was 90 pre-treatment. He is happy and well-looking. He is sexually active. What is the most likely cause of his hematuria:
a. cyclophosphamide-induced hemorrhagic cystitis
b. thrombocytopenia due to myelosuppression
c. chlamydial urethritis
d. urine infection
A
cyclophosphamide-induced hemorrhagic cystitis
18
Q
You are seeing a 1 wk old Chinese boy with 1 day of jaundice. His bili is 270 (mostly indirect), Hg 95 retics 9%, Mom is AB+ and he is B+. He otherwise looks well. What is the diagnosis?
a. Sepsis
b. Thalassemia
c. G6PD def
d. ABO incompatibility - Mom is AB+
A
g6pd
19
Q
Schwachman diamond baby. Which vitamin level would be normal?
a. Vit A
b. Vit E
c. Vit B12
d. Bit D
A
vit b12
Autosomal recessive condition, disorder of ribosomal function. Characterized by pancreatic insufficiency (diarrhea, fat malabsorption, FTT in infancy), neutropenia, variable cytopenias, skeletal dysplasia, immunodeficiency (B and T cell), risk of myelodysplasia and AML
Pancreatic insufficiency treated with pancreatic enzymes and fat soluble vitamins, may improve over time
Fat soluble vitamins: ADEK
20
Q
3 yo Sickle cell, with fever cough, tachypnea, unwell. What do you need to rule out 1st?
a. Acute chest crisis
b. PE
c. Pneumonia
d. Asthma
A
Acute chest crisis
21
Q
2 year with normal history but found to be pale on exam. Labs show normocytic normochromic anemia. Smear normal. What next test?
a. Bone marrow
b. Osmotic fragility
c. Ferritin
d. Hg electrophoresis
A
BMA
22
Q
A 2 yo boy presents with pallor. He has been drinking 1L of milk each, but does each a varied diet. Bloodwork is as follows: Hb 49, MCV 80. RDW is 14%. Peripheral blood smear: normal. Hemoglobin electrophoresis: Hb A and Hb S [exact wording on exam]. Which of the following conditions is most likely?
Transient erythroblastopenia of childhood
Iron deficiency anemia
3. Sickle cell disease
A
Transient erythroblastopenia of childhood:
- transient or temporary red cell aplasia (temporary cessation in erythrocyte production).
Pure red cell aplasia is distinguished by anemia with reticulocytopenia. Normally bone
- usually in children older than 6 months
- MCV is normally not elevated at the time of diagnosis, but then may become elevated by the time of recovery
- Bone marrow aspirate should show signs of decreased or absent erythroid precursors.
**It would be very helpful to know if the reticulocyte count was increased or decreased to increase suspicion of this diagnosis
23
Q
Mother brings her 6 month old baby boy to you office. He has had two episodes of AOM since birth. He also has eczema and some bloody stool. He also had prolonged bleeding after his circumcision. What is the likely diagnosis?
1) Wiskott Aldrichth
A
WATERBOY (wiskot aldrich, thrombocytpenia, recc infxn, x link
24
Q
A 5 day old baby is seen in your clinic. Pregnancy was unremarkable, no ABO incompatibility. The baby is breastfeeding. The unconjugated bilirubin is 200. What do you do? (**note: they did NOT provide the phototherapy curves!)
Start phototherapy
Reassure
Check for G6PD
A
reassure
REMMEBER 250 is the plateau for phototherapy (less is fine at 3 d)
Breast milk jaundice has been traditionally defined as the persistence of “physiologic jaundice” beyond the first week of age. It typically presents after the first three to five days of life, peaking within two weeks after birth, and progressively declined to normal levels over 3 to 12 weeks
25
9) A newborn baby with platelets of 10. Mom also has low platelets. What is the diagnosis?
Autoimmune thrombocytopenia
Alloimmune thrombocytopenia
TAR
Autoimmune thrombocytopenia is mediated by maternal antibodies that react with both maternal and fetal platelets. This occurs in maternal autoimmune disorders, including immune thrombocytopenia purpura (ITP) and systemic lupus erythematosus (SLE).
NAIT - alloimmunotherapy (father ag)
TAR (Thrombocytopenia-absent radius syndrome): Autosomal recessive disorder characterized by severe thrombocytopenia (platelets 10-30,000), bilateral absent radii although the thumbs are always present. Other limb abnormalities include hypoplasia/absent ulna, absent/abnoraml humerus. CHD: TOF or ASD occurs i
26
A baby is born precipitously at home to parents who both have type 3 von Willebrand disease. The baby is found to have an intraventricular hemorrhage. Which of the following would be the most appropriate treatment?
a. Cryoprecipitate
b. FFP
c. Factor VIII/vWF concentrate
d. DdAVP
Factor VIII/vWF concentrate
Because type 3 VWD is caused by a lack of VWF, treatment with VWF-containing concentrates is required. Treatment of vWD type 2 and 3 require VWF containing concentrates similar to the treatment of hemophilia.
Type 1: low levels of vWF < 30, most common form of vWD. Can be treated with desmopressin with exception of type 1C, which arises from increased clearance of vWF (desmopressin likely to be ineffective). Desmopressin increases the amount of circulating vWF from storage.
Type 2 - poor function of vWF
UTD: In type 3 VWD hemarthrosis, muscle hematoma, oral cavity bleeding and epistaxis were seen (in order of increasing frequency)
27
15 year old girl has cervical lymphadenopathy which has been waxing and waning over the last 8 months. She had a CXR which showed a widened mediastinum. Which of the following is the most appropriate next test?
a. Cervical node excision
b. TST
c. CT chest
d. Bartonella serology
CT chest
28
A 12 year old boy presents with an X-ray (described, no picture given) of mottled bone on right rib with new bone forming on top. What is the most likely diagnosis?
a. Ewings
b. Osteosarcoma
c. Osteochondroma
Ewings
Ewing’s sarcoma: “Moth eaten” appearance, created by finely destructive lesions becoming confluent over time. Periosteal reaction produces layers of reactive bone creating an “onion peel” appearance.
Osteogenic sarcoma- Codman's triangle (an incomplete response of host periosteal bone) The associated soft tissue mass is variably ossified in a radial or "sunburst" pattern.
Osteochondroma: An osteochondroma (osteocartilaginous exostosis) is a cartilage-capped bony spur arising on the external surface of a bone
29
A baby’s platelets are found to be low on day of life 2. His mother’s platelets are normal. What is the most likely diagnosis?
a. Maternal ITP
b. NAIT
NAIT
30
A young boy presents with a normochromic, normocytic anemia. He is stable. What is your next course of action?
a. Start iron supplementation
b. Refer to heme for BMA
c. Follow up in 2 weeks
fu 2 weeks
31
Child with sickle cell disease presenting with fever and respiratory symptoms and a change on X-ray. Management?
a. Start 2x maintenance IV fluids
b. Antibiotics
c. Transfuse pRBCs
abx
ACS defined as new radiodensity on chest X ray plus any 2 of the following: fever, respiratory distress, hypoxia, chest pain and cough
Most common organisms are S. pneumoniae, Chlamydia, Strep pneumoniae
All episodes should be treated promptly with a macrolide and third generation cephalosporin.
32
n Asian baby presents at 12 hours of life with jaundice. Mother is O+, the baby is A+. The bilirubin is 200. What is the diagnosis?
a. ABO incompatibility
b. G6PD -
c. Rh incompatibility -
d. Physiological jaundice -
A- ABO
g6bd is possible too
33
You are treating a 7 year old girl with ALL for a clot from her central line. Despite increasing doses of heparin you are not getting a therapeutic level. What is the cause for this?
a) antithrombin III deficiency
b) protein C deficiency
c) Factor V Leidin
d) Factor VIII deficiency
a) antithrombin III deficiency
34
7 year old Child with anemia hgb 40, BMA shows arrested erythroid precursors. Cause?
a) TEC
TEC
transient hypoplastic anemia in previously healthy children 6mo-3yrs, suppression of erythropoiesis thought to be immune mediated, often follows viral illness, virtually all kids recover within 1-2 mo
35
Chemotherapy that causes low sodium and vomiting
a) Vincristine
b) Cyclophosphamide
c) Anthracycline
b) Cyclophosphamide (alkylating) SIADH, hemorrhagic cystitis, encephalopathy. Late: infertility, cardiac dysfunction, pulmonary fibrosis
vincristine can cause
SIADH (4-6 days), peripheral neuropathy (constipation, hyporreflexia)
36
What is a side effect of doxyrubicin
a) cardiopmyopathy or arrythmia
b) mucositis
c) n/v
d) alopecia
e) all above
E
its an anthracycline
37
Sickler with dactylitis, Hgb 95, which will be associated with worse prognosis
a) Anemia
b) Dactylitis
Dactylitis before 1 year of age was identified as one of three prognostic factors used to predict severe outcome (frequent VOCs, frequent acute chest syndrome [ACS], acute stroke, or death) i
38
Kid with 2.5cm supraclavicular node. 8kg wt loss in past 6mos. What do you do first?
a) IV Piperacillin - no fever
b) IV fluids and allopurinol
c) IV methypred - not without ruling out TB
d) Isoniazid - would test for TB first
b) IV fluids and allopurinol
39
Kid with Burkitt’s and TLS. Urine pH 7.0. What’s next.
Hemodialysis
Potassium - no, TLS is characterized by hyperkalemia, hyperurecemia, high PO4, low Ca
ACE inhibitor
Rasbiricase
Rasbiricase
decreasing uric acid formation, allopurinol does not reduce the preexisting serum uric acid. Thus, for patients with preexisting hyperuricemia (serum uric acid ≥7.5 mg/dL [446 micromol/L]), rasburicase is the preferred hypouricemic agent
40
An 11 month old presents with a scaly rash all over, especially in the diaper area. He also has exopthalmos and HSM. Xrays show bony lucencies on the scalp. What is the likely diagnosis?
Neuroblastoma
Langerhans cell histiocytosis
ALL
LCH
polyostotic disease can involve the skin, liver or other organs. Usually occurs in the first 3 decades of life, more common in boys age 5-10 years. The skull is most typically affected but any bone can be affected. Patients usually present with local pain and swelling. Marked tenderness and warmth often are present in the area of the affected bone. Radiolucent lesions
41
```
A Child has a distended abdomen. An ultrasound shows an adrenal mass and hepatomegaly. Which of the following tests will make the diagnosis?
MIBG Scan
Serum AFP
Urine HVA/VMA
Abdo/Chest CT
```
urine HVA/VMA
42
New born baby born with refractory hypoglycemia and attached photo. What is he most at risk for?
Hirschprung’s disease
Wilm’s tumour
Hypothyroid
Wilm’s tumour
ALSO BWS+
Wilms tumor and hepatoblastoma, but also neuroblastoma, adrenocortical carcinoma, and rhabdomyosarcoma
43
```
Why do we irriadiate blood given to prems?
Decrease CMV
Decreased GVHD
Sterilize RBC
Decrease hemolytic reactions
```
dec GVHD
44
Baby with petichiae. Plt 12. After transfusion, Plt are 16. Mom’s CBC normal. What is best management?
PLA1 negative platelets
IVIg
PLA1 neg plt
45
Newborn with trisomy 21. High WBC, thrombocytopenia, anemia, HSM, petechiae. What does he have?
CMV
Sepsis
Transient myeloproliferative
Transient myeloproliferative
46
```
given this haemoglobin electrophoresis, what is the most likely diagnosis?
hgbA none
hgb A2 2%
hgb F 75%
hgb S 25%
```
A. Sickle cell trait
B. Sickle cell disease
C. Beta thalassemia
D. Alpha thalassemia
sickle cell disease
47
```
2 yo girl from Mediterranean background. What values are most representative of thalassemia minor?
Hgb 100 MCV 75 RBC 2.61
Hgb 100 MCV 60 RBC 4.81
Hgb 80 MCV 75 RBC 2.81
Hgb 80 MCV 60 RBC 3.2
```
Hgb 100 MCV 60 RBC 4.81
Beta Thal Minor:
Hematocrit >30 percent, and MCV <75 fL.
RDW normal in thalassemia, increased in iron deficiency
Mentzner Index: MCV/RBC for thalassemia is < 13, for iron deficiency > 13
48
A boy with Hemophilia A who is managed at home with recombinant Factor VIII presents with increasing episodes of bleeding and hemarthrosis of the ankle despite medication compliance. Your next step:
a) Check FVIII and inhibitor levels
b) Add DDAVP to the current regime
c) Try another FVIII product
d) Manage ankle with NSAIDs and rest.
a) Check FVIII and inhibitor levels
49
A 10 year old with sickle cell anemia presents with fever and respiratory distress. On CXR there is a new infiltrate. Your next most important step:
a) Order type and screen and transfuse
b) IV antibiotics
c) IV hydration at 2x maintenance
d) Start hydroxyurea
IV antibiotics
50
An 7 day old term Asian baby presents with jaundice. He looks well. Mom’s blood type AB+, baby B+. Hgb 104, bilirubin 207, retics 8%. Most likely etiology:
a) Sepsis
b) ABO incompatibility
c) G6PD
d) Thalassemia
g6pd
51
Baby with suspected trisomy 21 with a petechiael rash, high WBC, anemia, and thrombocytopenia. On exam, has hepatosplenomegaly. What is the most likely reason for his presentation? [repeat]
Sepsis
CMV infection
Transient myeloproliferative disorder
Transient myeloproliferative disorder
52
Child (age?) with ataxia, diplopia and headaches. What is the most likely dx?
a. brainstem glioma
b. cerebellar astrocytoma
c. craniopharyngioma
d. ependymoma
b. cerebellar astrocytoma
triad of headache, nausea, and vomiting as well as papilledema is associated with midline or infratentorial tumors. Disorders of equilibrium, gait, and Torticollis may occur in cases of cerebellar tonsil herniation. Blurred vision, diplopia, and nystagmus also are associated with infratentorial tumors.
Supratentorial tumors are more commonly associated with lateralized deficits, such as focal motor weakness, focal sensory changes, language disorders, focal seizures, and reflex asymmetry.
53
Picture given of large hemangioma over V1 to scalp and upper eyelid distribution with bluish tint. Baby presents with this facial lesion and high output CHF. Apart from thrombocytopenia what else would you expect to see on CBC or smear:
a. Neutropenia and mild anemia
b. Normal INR/PTT
c. Schistocytes
d. High Fibrinogen
c. Schistocytes
54
Picture of a baby sucking on a pacifier and looking relatively content. Severe thrombocytopenia. Has a large lesion (looks kinda like the picture here) overlying his left forehead and eyelid (not a port-wine stain). What is the most likely finding on labs?
a. normal INR and PTT
b. elevated fibrinogen - would see low fibrinogen
c. schistocytes and RBC fragments on smear
d. neutropenia and anemia
. schistocytes and RBC fragments on smear
giant hemangioma with localized intravascular coagulation causing thrombocytopenia and hypofibrinogenemia is called Kasabach-Merritt syndrome.
55
Teen with weight loss, generalized lymphadenopathy including supraclavicular node, and palpable spleen tip. More fatigue recently. Afebrile. WBC 10, Hgb 120, Plt 150. Normal Monospot. What is the next step in evaluation:
a. BM aspirate
b. Chest Xray
c. Excise node
d. ANA
CXR
56
Wilm’s tumour is most associated with:
a. Tuberous sclerosis
b. Fragile X
c. Angelman’s
d. Beckwith Weidemann
beckwith weideman
57
sickle cell most common neurologic sequelae
a. Silent stroke
b. Clinical Stroke
c. Seizures
silent stroke
58
Sickle cell anemia, which is true about strokes?
a. usually subclinical strokes
b. clinical stroke
subclinical stroke
59
Teenage girl with SSD. Acute fever, jaundice, abdo pain. Suspect cholecystits, confirmed with U/S. After treating for one week, what do you suggest?
a. Cholecystectomy
b. Ursodiol
c. ERCP
a. Cholecystectomy
a. Cholecystectomy
60
13yo, obese with HbSS and RUQ pain, tenderness, fever, guarding and jaundice. U/S shows multiple gallstones, dilated CBD, inflammed walls. You diagnose acute cholangitis and admit for hydration and triple antibiotics. After the acute process is treated, what do you suggest:
a. transfusion therapy
b. Ursodiol
c. Cholecystectomy
d. Nothing
a. Cholecystectomy
61
A 9 month old ex-32 weeker takes 40 oz of homo milk per day. Hgb is 60, MCV 50. She is treated with Fe 4 mg/kg/day for one month. On repeat testing, her Hgb is 62, MCV is 50, and her retics are 0.01. What to do next?
a. BMA
b. verify compliance
c. Hb electrophoresis
d. Jejunal biopsy
verify compliance
62
Iron deficiency anemia, what will you find on exam:
| a. Pica
PICA
63
15 year old girl with periods for a couple of years. Epistaxis and menorrhagia. What to check for?
a. von Willebrands
b. Hemophilia C
c. Factor V Leiden mutation
hemophilia A/B Xlinked
Hemophilia C - plasma thrombin antecedent (PTA) deficiency or Rosenthal syndrome, factor XI deficiency
von willebrands
common congenital bleeding disorder, autosomal dominant, typically involves mucous membranes, skin, surgical, menstrual bleeding (if severe disease, may also have profound factor VIII def and hemarthrosis). Caused by deficiency of vWF (quantitative - type 1=partial, 80% of people, or type 3=absolute; qualitative - type 2=dysproteinemia).
Treatment is desmopressin in type 1 and 2, if need high levels of vWF then Humate P (vWF-containing concentrate), don’t use cryoprecipitate (not virally attenuated)
64
Which cancer will most likely have bone marrow infiltration?
a. Wilms
b. neuroblastoma
c. hepatoblastoma
d. ?bone CA of some sort ( I think osteosarcoma)
neuroblastoma
65
18 month old male (13kg) with history of excessive milk intake presents on routine physical exam with pallor Subsequent bloodwork reveals Hb 45, MCV 56. What would be the best management?
a. Limit milk to 500 cc/day
b. Start elemental iron 60mg PO tid
c. Give PRBC transfusion 130cc
d. Change to protein hydrosylate formula
limit 500cc/d
66
Hb 48 in an 11 m.o. M with tachycardia, pallor, excess milk intake.
a. restrict milk intake to < 500 cc/day
b. Fe TID at 15 mg/kg
c. Transfuse 10 cc/kg blood
d. Change to protein hydrolysate formula
transfuse 10cc/kg blood
67
An otherwise healthy girl presents with pallor. Her iron, and ferritin levels are normal. Her bloodwork shows a Hb of 70 with an MCV of 50. What study is most likely to give you her diagnosis?
a. Bone marrow aspirate
b. Hemoglobin electrophoresis
hgb electrophoresis
68
What is the advantage of using leukoreduced white cells?
a. Decreases hemolytic reactions
b. Decreases infectious complications -
c. Decreases febrile transfusion reactions
dec infxn complications
Leukoreduction" refers to the removal of white blood cells (WBCs) from blood products by highly efficient filters that reduce the number of WBCs by more than 99.9 percent, generally to <1 x 106 WBCs per red cell unit
The use of leukoreduced RBCs reduces the risk of the following adverse consequences of transfused WBCs:
69
A 7 year old boy who has had recent personality changes, decline in school performance and visual changes. Which is the first diagnosis to rule out?
a. Brain tumour
b. DM
c. ADHD
d. Depression
brain tumor
70
8 year old health child with supraclavicular lymph node. What to do?
a. excise
b. PPD
c. bartonella serology
d. EBV
excise
71
What is a risk factor for child leukemia?
a. parent treated for leukemia
b. in utero radiation
c. maternal alcohol
d. NF type 1
NF1
Down syndrome, Fanconi anemia, Bloom syndrome, Diamond-Blackfan anemia, Schwachman-Diamond syndrome, Kostmann syndrome, Neurofibromatosis type 1, Ataxia-telangiectasia, Severe combined immune deficiency, Paroxysmal nocturnal hemoglobinuria, Li-Fraumeni syndrome
72
Which of the following is an indication for bone marrow biopsy in a child with ITP who has platelets of 12 000?
a. ANC <1000
b. Previous use of steroids
c. Hb <115 - can get mild anemia due to bleeding, if unexplained will need BMA
d. Fever > 39
anc <1
73
16 y.o. boy undergoing treatment for non-Hodgkin’s lymphoma. Forty-eight hours after his last chemotherapy, he develops mild dysuria and hematuria. Platelet count was 90 pre-treatment. He is happy and well-looking. He is sexually active. What is the most likely cause of his hematuria:
a. cyclophosphamide-induced hemorrhagic cystitis
b. thrombocytopenia due to myelosuppression
c. chlamydial urethritis
d. urine infection
cyclophosphamide-induced hemorrhagic cystitis
74
You are seeing a 1 wk old Chinese boy with 1 day of jaundice. His bili is 270 (mostly indirect), Hg 95 retics 9%, Mom is AB+ and he is B+. He otherwise looks well. What is the diagnosis?
a. Sepsis
b. Thalassemia
c. G6PD def
d. ABO incompatibility
g6pd def
75
Schwachman diamond baby. Which vitamin level would be normal?
a. Vit A
b. Vit E
c. Vit B12
d. Bit D
b12
Schwachmann Diamond
Autosomal recessive condition, disorder of ribosomal function. Characterized by pancreatic insufficiency (diarrhea, fat malabsorption, FTT in infancy), neutropenia, variable cytopenias, skeletal dysplasia, immunodeficiency (B and T cell), risk of myelodysplasia and AML
Pancreatic insufficiency treated with pancreatic enzymes and fat soluble vitamins, may improve over time
76
3 yo Sickle cell, with fever cough, tachypnea, unwell. What do you need to rule out 1st?
a. Acute chest crisis
b. PE
c. Pneumonia
d. Asthma
ACS
77
2 year with normal history but found to be pale on exam. Labs show normocytic normochromic anemia. Smear normal. What next test?
a. Bone marrow
b. Osmotic fragility
c. Ferritin
d. Hg electrophoresis
BM
78
14 yo male with constitutional symptoms, weight loss, generalized lymphadenopathy including supraclavicular. Hb 120, WBC 10, Plt 150. Splenomegaly present. What is your initial investigation?
a. CXR
b. Abdominal CT
c. Bone marrow
d. Tb skin test
CXR
79
What is the number one cause of complications in beta thalassemia major?
a. Iron overload
b. Megaloblastic anemia
c. Cardiomyopathy
d. Poor hematopoeisis
Iron overload is caused by ineffective erythropoiesis which stimulates excessive iron absorption, and then is further exacerbated by chronic transfusions
80
Child with history of multiple acute chest syndrome episodes is at most risk for which of the following:
a. Stroke
b. Gallstones
c. Asthma
d. Priapism
asthma and stroke
81
SCD patient: which of the following would be most suggestive of stroke?
a. Fatigue
b. Sudden decrease in Hb
c. Decrease in school performance
dec school perf
82
In a child with sickle cell disease, what would be a sign of stroke
a. Sudden onset anemia
b. Painful limp
c. fatigue
d. Poor scholastic performance
school perf
83
You are counseling a mother of a SCD patient who will be requiring blood transfusions. Which of the following would she be most likely to receive in a blood transfusion?
a. Hep B
b. Hep C
c. Parvovirus B19
d. Variant of Creutzfeldt Jacob disease
parvo
Hep B (1 in 1.2 mill)
b. Hep C (1 in 5mill)
c. Parvovirus B19 (1 in 5000)
d. Variant of Creutzfeldt Jacob disease (1 in 10 mill)
84
What picture is most consistent with thalassemia minor?
a. Hb 100 MCV80 RBC 2.81
b. Hb 100 MCV 60 RBC 4.81
c. Hb 60 MCV 80 RBC 4.81
d. Hb 60 MCV 60 RBC 3.71
Hb 100 MCV 60 RBC 4.81
Beta Thal Minor:
Hematocrit >30 percent, and MCV <75 fL.
RDW normal in thalassemia, increased in iron deficiency
Mentzner Index: MCV/RBC for thalassemia is < 13, for iron deficiency > 13
85
4-year old of Mediterranean descent. Lab findings most consistent with thalassemia minor?
a. Hb 100 MCV 60 RBC 4.8
b. Hb 100 MCV 75 RBC 4.3
c. Hb 80 MCV 75 RBC 3.3
d. Hb 60 MCV 50 RBC 2.2
Hb 100 MCV 60 RBC 4.8
Beta Thal Minor:
Hematocrit >30 percent, and MCV <75 fL.
RDW normal in thalassemia, increased in iron deficiency
Mentzner Index: MCV/RBC for thalassemia is < 13, for iron deficiency > 13
86
3 y.o. Chinese boy sees you in the emergency room after falling off his bicycle 24 hours ago. On exam, you see a swollen right knee and a yellow bruise on the anterior chest. What would explain this?
a. Chinese herbal remedies
b. Hemophilia
c. Child abuse
d. von Willebrand disease
hemophilia
87
6-year Chinese boy presents after falling off his bike with a single bruise on his chest and a swollen, painful left knee. What is the most likely diagnosis?
a. ITP
b. Child abuse
c. Hemophilia
hemophilia
88
3 year old girl with fever, arthralgia, lethargy x 10d
• P/E: lymphadenopathy, moderate HSM, no
obvious arthritis but cries out in pain with
minimal examination
• WBC 9.5 Hgb 98 Plts 140, smear normal
What do you do next?
a) Bone marrow aspirate
b) Abdominal ultrasound
c) EBV titers
d) Refer to rheumatology
BMA
89
```
P/E: unwell, generalized petechiae, HSM
• WBC 8.1, HgB 65, Platelets 41, ANC 0.5
How would you triage this patient?
a) Book into your next office appointment
b) Call Rheum to expedite the consult
c) Request same day office visit
d) Advise to go to ER
```
go to ED
90
A 3-year-old girl has had persistent fever and sore
throat for several days despite antibiotic therapy.
• P/E: ulceration of her tonsils and oropharynx,
generalized lymphadenopathy, splenomegaly,
and purpuric lesions on her lower extremities.
• WBC 25.6, hemoglobin 80, platelets 30.
What is the most likely diagnosis?
What is the most likely diagnosis?
a) Infectious mononucleosis
b) Acute lymphoblastic leukemia
c) Idiopathic thrombocytopenic purpura
d) Aplastic anemia
e) Lymphoma
b ALL
91
at risk of ALL
1. Trisomy 21
2. Neurofibromatosis Type 1
3. Ataxia Telangiectasia
4. Fanconi anemia
5. Li-Fraumeni Syndrome
6. Bloom syndrome
7. Noonan syndrome
92
Poor prognosis in ALL is suggested by:
a) Female
b) Age< 1 year
c) CALLA positive (CD10)
d) Mediastinal mass
e) Splenomegaly
b) Age< 1 year
93
what cytogenic favourable for ALL
1) ph22
2) hyperdiploid
3) MLL gene rearrangement
4) hypodiploid
HYPERDIPLOID
94
A 7 year old girl presents in Northern Ontario
with fever, pallor and hepatosplenomegaly.
Her CBC shows a WBC of 352 x 10^9, HGB 52
g/L, PLT 52 x 10^9. The physician there would
like to transfer for further care.
Your recommendations prior to air
transfer include:
Your recommendations prior to air transfer
include:
a) Stat lytes and NS at 1.5 x maintenance. Transfuse
PRBCs. Allopurinol
b) Stat lytes and NS plus 20meq KCL/L at 2 x
maintenance. Transfuse PLTs
c) Stat lytes and LR at 1 x maintenance. Antibiotics.
Allopurinol
d) Stat lytes and NS at 1.5 x maintenance. Review smear.
Antibiotics. Allopurinol
e) Send by air ambulance immediately
d) Stat lytes and NS at 1.5 x maintenance. Review smear.
Antibiotics. Allopurinol
dont want to give transfusion of blood
CAN give plt as doesnt increase viscosity
95
A patient presents with a supraclavicular lymph
node. Next steps?
a) CXR
b) Excisional biopsy
c) Antibiotics
d) TB skin test
cxr
96
15 yo male in resp. distress with first episode of
wheeze in ER. Resolved with ventolin and IV
methylpred. One week later, CXR done due to
ongoing symptoms. Mediastinal mass seen.
What is the most likely diagnosis
a) ALL
b) Hodgkin lymphoma
c) Sarcoidosis
d) TB
hodgkin lymphoma
97
```
14 yo male with constitutional symptoms,
weight loss, generalized lymphadenopathy
including supraclavicular.
Hb 120, WBC 10, Plt 150. Splenomegaly present.
What is your initial investigation?
a) CXR
b) Abdominal CT
c) Bone marrow aspirate
d) TB skin test
```
CXR
98
A 15 year old is brought in for evaluation of ‘swollen
lymph glands’ and a concern for cancer. Of the
following points on history, which is most suggestive
of a malignant rather than infectious cause?
a) Aphthous stomatitis
b) Chronic cough
c) Lymph node enlargement < 4 weeks
d) Age < 4 years
e) Weight loss
weight loss
<4yo (not common to have lymphoma)
99
You proceed to perform a careful physical exam of the patient. You palpate each region of the body for lymph nodes.
An enlarged lymph node in which of the following locations would be most concerning for malignancy?
a) Anterior cervical
b) Inguinal
c) Posterior cervical
d) Submandibular
e) Supraclavicular
supraclav
100
```
14 year old with nodes + sweats
• Previously healthy
• 2mo h/o progressive neck node enlargement
▫ Positive serology for Bartonella henselae
▫ Treated with 2 courses of antibiotics
▫ Onset of drenching night sweats in past 2 weeks
What is your next step?
a) Refer for fine needle aspiration
b) Repeat trial of antibiotics
c) Send for CXR
d) Request toxoplasmosis serology
e) Throat swab in clinic
```
CXR
if NEG - consider exisional bx
FNA cannot do as architecture affected and also can seed
101
10 yr old boy with SOB. CXR reveals a large mediastinal mass and a moderate right-sided pleural effusion. The pleural fluid contains numerous lymphoblasts that are
TdT-positive and express T-cell antigens. On bone marrow aspiration, lymphoblasts with identical
characteristics represent 12% of the total cells.
What is the diagnosis?
a) Hodgkin lymphoma
b) T cell ALL
c) Burkitt lymphoma
d) Lymphoblastic lymphoma
e) neuroblasoma
D lymphoblastic lymphoma]
TDT = premature
lymphoma - nodal
usu hodgin kiddos are walking around and look well
102
Neck mass in a baby
6 month old, noted by mom to have a left neck mass. US showed a 4 cm mass near parotid gland. Referred to ENT. Mass noted to be decreasing in size. Repeat US 1 month later showed growth and
vessel encasement. What’s the next best investigation?
```
CT done – mass compatible with necrotic
adenopathy; neoplastic etiology could not be excluded given the interval growth
+ lytic lesions
thiink in babies
neuroblastoma ultimately
```
103
```
8 year old boy with 1d hx of acute abdominal pain and BRBPR. DIAGNOSIS
O/E: unwell, abdomen distended with palpable mass
Hgb 115
Plt 400
WBC 12.3 ANC 9.2
No BLASTS
K 5.5
Pho 3.3
Ca 1.8
Cr 90
Normal liver enzymes
LDH 2100
Urate 600
```
tls
from abdominal burkitts
104
An 8-year old boy with progressive enlargement of a right
axillary node, now tender, and daily fevers.
You discover that the boy has been playing with his family’s new kitten. You suspect the child may have a related infection and is
uncomfortable enough to treat. What is your preferred antibiotic?
a) Amoxicillin
b) Azithromycin
c) Doxycycline
d) Cephalexin
e) Penicillin
b) Azithromycin
105
4-year old presents with a 10-day hx of unilateral anterior
cervical lymph node enlargement. Temp 38.5C. Node is
2cm, warm and fluctuant. Mild pharyngeal erythema.
You suspect acute bacterial lymphadenitis. Of the
following, which are the most likely infectious agents to
cause lymphadenitis?
a) Bartonella and Staphylococcus
b) EBV and Staphylococcus
c) Mycobacterium TB and Staphylococcus
d) Staphylococcus and Streptococcus
e) Toxoplasma and Nocardia
Staphylococcus and Streptococcus
106
A 16-year old girl with 2 weeks of fatigue, fever, and sore throat.
On exam, you identify enlarged posterior and anterior cervical
nodes, and a palpable spleen tip.
She has mild thrombocytopenia (120 x 10^9/L). Of the
following, which would be the most specific test to confirm the
suspected diagnosis?
a) Bartonella henselae antibody titres
b) EBV antibody titres and monospot
c) Throat swab for GAS
d) HIV antibody titres
e) WBC differential and smear
EBV antibody titres and monospot
if they were 3 yo leaning towards malignancy
107
```
Which of the following are concerns with a
mediastinal mass?
a) Airway compromise
b) SVC syndrome
c) RVOT obstruction
d) Cardiac tamponade
e) All of the above
```
ALL
108
If you were worried about tumor lysis
syndrome, which of the following panel of
results is the one most likely to be seen?
a) Decreased K, increased HCO3, decreased P, increased Na, decreased
urate
b) Increased K, decreased HCO3, increased P, decreased Na, increased
urate
c) Increased K, decreased HCO3, decreased P, increased Na, decreased
urate
d) Decreased K, increased HCO3, increased P, decreased Na, increased
urate
e) Increased K, decreased HCO3, increased P, increased Na, increased urate
b) Increased K, decreased HCO3, increased P, decreased Na, increased
urate
109
```
12 yo boy with recent change in behavior,
irritability, daily headaches, change in vision.
What is the most important thing to
consider?
a) Brain tumor
b) Drug use
c) Psychiatric diagnosis
d) Migraines
```
brain tumor
110
```
A 6 yo girl presents with 2 month hx of head tilt,
diplopia, papilledema and ataxia
Where is the lesion?
What is the most likely diagnosis:
a) Cranipharyngioma
b) Dandy walker cyst
c) Brainstem glioma
d) Cerebellar astrocytoma (medulloblastoma)
```
d) Cerebellar astrocytoma (medulloblastoma)
with inc ICP and papiledema makes u think mass in cerebellum
couldve been brainstem
111
A 10 year old boy presents to your office with a history of waking in the mornings with a headache for the past month. He occasionally vomits with the headache.
Over the past week he has started to complain of double vision, and feels that he is unsteady on his feet.
What is your diagnosis?
cerebellar astrocytoma/posterior fossa tumor (like medulloblastoma)
112
```
What are some other physical exam
features that may be associated with this
child’s diagnosis? neuroblastoma
• Hypertension
• Abdominal mass
• Hyperreflexia
• Bone pain
• Bruising/petechiae
```
* Hypertension
* Abdominal mass
* Hyperreflexia
* Bone pain
* Bruising/petechiae
113
```
Opsoclonus-myoclonus-ataxia (OMA) is seen in
which of the following malignancies?
a) Leukemia
b) Rhabdomyosarcoma
c) Neuroblastoma
d) Medulloblastoma
```
neuroblastoma
114
```
Better prognosis with neuroblastoma is
associated with?
a) Female
b) High excretion of VMA/HVA
c) Age < 12-18 months
d) Normal blood pressure
e) Unilateral disease
```
age <18 mo good
115
A child presents with a distended abdomen. An
US shows an adrenal mass and hepatomegaly.
Which of the following tests will make the
diagnosis?
a) MIBG
b) Serum AFP
c) Urine VMA/HVA
d) CT chest/abdo
urine VMA/HVA
116
14 year old female presents with pain above right knee, worse at night and with activity. On exam there is no fever, but there is tenderness to palpation above
the knee. X-ray shows periosteal elevation and mottling of the
distal femur. The most likely diagnosis is:
a) Osteomyelitis
b) Osteosarcoma
c) Osteoid osteoma
d) Ewing’s sarcoma
e) Sub-periosteal hematoma
osteosarcoma
MORE DISTAL and in metaphysis
even tho it is mottled
Ewing is flat bone like ribs, spine, pelvis and is more midbone
117
```
A 12 year old boy presents with an X-ray
(described, no picture given) of mottled
bone on right rib with new bone forming
on top. What is the most likely diagnosis?
a) Osteosarcoma
b) Ewing sarcoma
c) Osteoid osteoma
d) Enchondroma
```
ewing
118
```
Why are PRBCs for neonates irradiated?
a. inactivate CMV
b. prevent hemolysis
c. sterilize the blood
?? none of the above
```
inactivate CMV
119
5. Child with ITP and no other concerning risk factors on exam or history. What will his platelet count be in one year from now?
a. Probably will be normal?
b. Depends on results of the current bone marrow
c. Depends on the treatment given
Probably will be normal?
PROGNOSIS
ITP: most often resolves spontaneously in 3 months.
Persistent ITP: 3-12 months.
Chronic ITP: >12 months = 20% of children
120
18 mo found to be pale on normal routine exam. Blood work shows Hb 80 (normocytic and normochromic), retics low, normal WBC and platelets. What additional test would you do?
a. Ferritin
b. Coombs
c. Bone marrow
d. Hb electrophoresis
BMA
121
A child is receiving a blood transfusion. She becomes febrile and develops chills. What is your management?
a. Continue with transfusion, give methylprednisolone
b. Stop transfusion, give steroids
c. Stop transfusion, run IV TKVO
d. Continue transfusion and slower rate
. Stop transfusion, run IV TKVO
122
9. What is the number one cause of complications in beta thalassemia major.
a. Iron overload
b. Megaloblastic anemia
c. Cardiomyopathy
d. Poor hematopoeisis
iron overload
123
Beckwith-Wiedeman syndrome U/S. A question about how frequently ultrasound should be done.
Abdominal ultrasound examination every three months until age eight years
Abdominal ultrasound examination every three months until age four years [45,78,82,83] and renal ultrasound including the adrenal glands every three months between ages four years and eight years [55,81,84].
•Measure serum AFP concentration every two to three months in the first four years of life for early detection of hepatoblastoma [82]. AFP serum concentration may be mildly elevated in children with BWS in the first year of life in the absence of a hepatoblastoma
124
Which of the following is present in tumour lysis syndrome?
a. hyponatremia
b. hypokalemia
c. hyperuricemia
d. hypophosphatemia
c. hyperuricemia
125
An 8 month old baby presents with an abdominal mass, thrombocytopenia, systolic murmur and a murmur over the liver. Ultrasound shows a 3 cm mass in the liver. What would you do?
a. DIC work-up
b. Bone marrow
c. Pulmonary imaging
d. Liver function tests
DIC
Tufted angioma and kaposiform hemangioendothelioma (KHE) are rare vascular tumors that typically occur during infancy or early childhood. KHE is a locally aggressive tumor that may involve superficial and deep soft tissues and, rarely, the retroperitoneum, mediastinum, and internal organs.
Complication: Kasabach-Merritt phenomenon - life-threatening coagulopathy characterized by severe thrombocytopenia and consumption of fibrinogen and other coagulation factors.
126
. 14 year old with AML who has failed three prior treatments. He now has a relapse. His parents want to continue with experimental treatment. He refuses. Which of the following is true
a. If the teen understand the risks and benefits, he may refuse
b. Parents should always decide
c. Health care team decides
d. Because he has agreed to treatment in the past, he should be treated
e. Must continue with treatment as the parents consented at the outset of therapy
f. Consult an ethics committee
a. If the teen understand the risks and benefits, he may refuse
127
8. 7 year old Jehovah’s witness is involved in MVA. Bleeding and has hemoglobin of 50 and is unstable.
a. Treat immediately
b. Try to talk to the parents to consent blood
c. Wait for a court order to be able to give the blood
treat immediatley
128
A child with a diagnosis of ALL begins to develop tumour lysis syndrome. What abnormality would you expect on blood work.
a. Hyperuricemia
b. Hypokalemia
c. Hypophosphatemia
d. Hypercalcemia
a. Hyperuricemia
129
13 year old boy has been wheezing (bilat) for 2 days and resp distress. This was his first episode of sneeze. He is treated in the emergency room with IV steroids and ventolin. He improves and is discharged. Later when you are reviewing the CXR, the radiologist informs you that he has a mediastinal mass/widened mediastium. The boy returns to hospital two weeks later. What was his likely diagnosis
a. Hodgkin’s lymphoma
b. Acute lymphocytic leukemia
c. Thymoma
d. Sarcoidosis
. Hodgkin’s lymphoma
130
You saw a 15 year old M with respiratory distress and bilateral wheeze in the ER. This was the first episode of wheeze. Resolved with IV methylprednisone and ventolin in ER and he was sent home. One week later the radiologist is reviewing the film and notices a widened mediastinum. What is the most likely cause?
a. Hodgkin’s lymphoma
b. ALL (? T-cell)
c. Sarcoidosis
Hodgkin’s lymphoma
131
7 year old boy with diplopia, head tilt and ataxia. Which is the most likely lesion?
a. Brainstem glioma
b. Cerebellar astrocytoma
c. Posterior fossa hemorrhage/hematoma
d. Craniopharyngioma
Cerebellar astrocytoma
132
Teenage sickler girl, sexually active has had previous transfusions. She presents looking pale and icteric, with abdominal pain. On exam her right upper quadrant is tender with no guarding. Her hemoglobin is 70, AST 60 and ALT 55. Her bilirubins are elevated. What is her diagnosis?
a. Liver vaso occlusive crisis
b. Acute cholecystitis
c. Hemachromatosis
d. Hep C
e. Fitz-High Curtis
f. Viral hepatitis
Acute cholecystitis
133
Teenager (or 7yo?) with Sickle cell disease presents with cough and fever. CXR shows pneumonia. She looks toxic, fully immunized. What do you treat her with
a. Cefuroxime and eryhtromycin
b. Ceftriaxone and erythromycin
c. Amoxicillin
b. Ceftriaxone and erythromycin
134
7yo with Sickle cell and pneumonia, fully immunized. What do you treat her with?
a. Cefuroxime and biaxin
b. Ceftriaxone and biaxin
ACS: Broad spectrum empiric coverage with a third generation cephalosporin (eg, cefotaxime or ceftriaxone) for bacterial coverage and a macrolide (eg, azithromycin or erythromycin) for coverage of atypical organisms (eg, mycoplasma and chlamydia) should be initiated immediately on admission. Severely ill - consider adding vanco.
135
Best prognostic sign in neuroblastoma
a. Age under 1 yo
b. Unilateral disease
age<1yo
Extent of metastatic spread = most important factor
Localized > localized with locoregional LN spread > mets to bone/BM
4S is separate from this (mets to liver and skin, but best prognosis)
Stage 4S (now called MS) = good prognosis
Metastatic disease in children younger than 12 months (new MS category = <18 months) with metastases confined to skin, liver, and/or bone marrow (BM involvement should be minimal; there should be NO cortical bone mets)
Cytogenetics/molecular genetics
MYCN amplification: poorer prognosis
1p deletion: poor prognosis
Trisomy 17q: poor prognosis
Hyperdiploidy: better prognosis in kids <2yo
136
Baby with abdominal mass, thrombocytopenia, II/VI LSB systolic murmur, murmur over liver (RUQ) as well. US showing 3 cm mass in liver (RUQ mass). What test should you do next?
a. DIC work-up
b. Bone marrow Bx
c. Pulmonary imaging
d. Liver function
DIC
Benign infantile hemangioendothelioma (IHH) is a rare neonatal benign vascular tumor usually located in the liver. Most of the lesions are diagnosed in the 1st few months of life. They may undergo spontaneous regression but can sometimes be life-threatening due to congestive cardiac failure (AV shunting) and/or consumptive thrombocytopenia and coagulopathy
137
Lab abnormalities in DIC include: consumption of platelets + coagulation factors, low fibrinogen, elevated D-dimer, microangiopathic hemolytic anemia, most have prolonged PT + INR
Abdominal mass in RUQ (documented on U/S as well). Systolic heart murmur at LSB and RUQ on exam. Conjugated hyperbilirubinemia. Low platelets. What test should you do next?
a. DIC work-up
b. Bone marrow biopsy
DIC workup
138
. Chinese boy fell off his bike yesterday. You are seeing him in ER and he has a large yellow bruise on his chest, and a tender swollen knee. What should you be most worried about?
a. Chinese herbal medicines
b. Child abuse (only thing about age of bruise is that if it is yellow it is >18 hours)
c. Hemophilia (or was it von Willebrand’s)
Hemophilia A
(factor VIII def) + B (factor IX def) are the most common and serious congenital coagulation defects
85% have A, 10-15% have B
X-linked recessive inheritance
No apparent racial predilection, appears in all ethnic groups
Hallmark of hemophilic bleeding = hemarthroses (may be induced by minor trauma or be spontaneous)
139
12 year old M with recent change in behavior, irritability, daily headaches and a change in his vision. What is the most important thing to consider?
a. Brain Tumour
b. Drug use
c. Psychiatric Diagnosis
brain tumor
140
40. 9 month old ex 32 weeker takes 40 oz of homo milk per day. Hgb is 60, MCV 50. She is treated with Fe 4mg/kg/day for one month. On repeat testing her Hgb is 62, MCV is 50, and her reticulocytes are 0.01. What to do next:
a. BMA
b. Verify compliance
c. Hb electrophoresis
d. Jejunal biopsy
Verify compliance
infants <12 months old SHOULD NOT consume cow’s milk. Infants who are not breastfed should only be given iron-fortified formula. If exclusively breastfed should have diet supplemented with iron (usually cereals) starting at 4-6 months. After 12 months, milk should be limited to <12-24 oz/day.
141
Which of the following is most likely to be seen in a child with Thalassemia minor?
a. Hgb 100, MCV 75, RBC 4.25
b. Hgb 100, MCV 60, RBC 4.81
c. Hgb 60, MCV 75, RBC 2.5
d. Hgb 60, MCV 60, RBC 2.5
b. Hgb 100, MCV 60, RBC 4.81
Individuals with beta thalassemia minor may have mild anemia; most are asymptomatic but exhibit marked microcytosis that can be mistaken for iron deficiency.
The RBC count is increased in thalassemias. This is especially true in thalassemia major and intermedia, but it may also occur in thalassemia minor. RBC count may also be normal in thalassemia minor.
*Normal RBC range in children = 4.5 - 5.1 (Lab Reference Values Sheet)
142
117. Child w/ supraclavicular node 1.5 x 2 cm, firm non-tender, mobile and no erythema. Do what?
a. excision biopsy
b. ppd
c. skin test for atypical mycobacteria
excision
143
16 yo girl with SCD presents with cough and fever. CXR reveals LLL pneumonia. She looks toxic. How do you treat?
a. cefuroxime and erythromycin
b. amoxicillin
c. erythromycin
d. Ceftriaxone
d. Ceftriaxone (all references would add macrolide as well)
Management
Prevention: incentive spirometry, ambulation, cautious use of IV fluids in inpatients
Testing: CXR, blood culture, NP swab, CBC
Monitoring: continuous pulse oximetry
Treatment: Empiric antibiotics (3rd generation cephalosporin + macrolide), ongoing respiratory therapy (chest physio + incentive spirometry), adequate pain management, fluid management, supplemental oxygen if <90%, blood transfusion PRN
144
Chinese boy brought in after a fall off his bike yesterday. He has a bruise on his chest and his right knee is swollen. You worry about:
a. hemophilia A
b. von Willibrands
c. thrombocytopenia
d. child abuse
e. use of herbal remedies
hemohplia
145
Child with SCD. What is he NOT at risk for?
a. H. flu
b. S pneumonia
c. N meningitides
d. S epidermidis
S epidermidis
146
At risk of infection with encapsulated organisms. Recurrent splenic infarction leads to functional asplenia in most patients by 5 years old
Child with sickle cell. Least likely invader:
a. pneumococcus
b. meningeococcus
c. HIB
d. Staph aureus
staph aures
147
What is NOT necessary to ask the mother of a 9mo old presenting with anemia?
a. duration of pregnancy
b. folate and B12 consumption during pregnancy
c. perinatal asphyxia
d. history of delayed cord clamping or perinatal hemorrhage
folate and B12 consumption during pregnancy
148
7 yo boy, previously well. Post tonsillectomy bleeding. Increased PTT, normal platelets, normal factor levels. What do you do?
a. Amicar (aminocaproic acid) - similar to tranexemic acid (MILD BLEEDS)
b. DDAVP (MILD BLEEDS)
c. factor VIII
d. Cryoprecipitate (IF SEVERE BLEEDING)
Von Willebrand disease and platelet function defects are the most common hematologic disorders leading to perioperative bleeding.
Patients with VWD have a normal prothrombin time (PT), and the activated partial thromboplastin time (aPTT) may be normal or prolonged, depending on the degree of reduction of the factor VIII level.
149
3y old with pallor, leg pain and fatigue X3w. Exam reveals tired pale anicteric child with HSM. No effusion or warmth/erythema of the legs. Most likely to confirm diagnosis?
a. BMA
b. Abdominal US
bma
Leukemia diagnosis strongly suggested by peripheral blood findings that indicated bone marrow failure. Bone marrow should be examined promptly to establish the diagnosis. (Nelsons)
150
Poor prognosis with histiocytosis:
a. Pancytopenia
b. Lymphadenopathy
c. Chronic lung disease
d. Vertebral body involvement
a. Pancytopenia
prominent proliferation or accumulation of cells of the monocyte-macrophage system of bone marrow origin. Includes LCH, HLH, Rosai-Dorfman, malignant histiocytosis, acute monocytic leukemia.
151
. 4y has a chest xray done for a different reason, but it shows an asymptomatic solid circular lesion in the anterior mediastinum:
a. Ganglioneuroma
b. Neuroblastoma
c. lymphomac
d. teratoma
e. mets from Wilms tumor
teratoma
ANTERIOR: lymphomas, thymomas, teratomas, angiomas, lipomas, and thyroid tumors.
MIDDLE: lymphoma, metastatic cancer, infection-related lymphatic lesions, malignancies that extend directly from the abdomen, pericardial cysts, bronchogenic cysts, esophageal lesions, and hernias.
POSTERIOR: neurogenic tumors such as neurofibromas, neuroblastomas, ganglioneuroblastomas, neurilemomas, ganglioneuromas, enterogenous cysts, thoracic meningoceles, and malignancies such as Ewing sarcoma (either osseous or extraosseous), lymphoma, and rhabdomyosarcoma.
152
A screening CXR done on a 4y reveals a posterior mediastinal mass. DX?
a. lymphoma
b. Teratoma
c. neuroblastoma
d. TB
A screening CXR done on a 4y reveals a posterior mediastinal mass. DX?
a. lymphoma
b. Teratoma
c. neuroblastoma
d. TB
153
A 9y old boy presents to ER department with unexplained bruising for the past 24 hours. There is a history of previous bruising or excessive bleeding. The patient has been very healthy during the past year. The patient is looking well and shows no signs of acute distress. A complete CBC has the following findings. Hg 115, WBC 10,500 and platelet count is 45,000.Large platelets are seen on the smear. Treatment:
a. IV immunoglobulin
b. no specific treatment is needed at this time
c. oral prednisone
d. platelet transfusion
b. no specific treatment is needed at this time
Thrombocytopenia with large platelets, mild anemia, normal WBC.
Typical of ITP - can get a mild anemia; if symptoms have been ongoing for a few days, large platelets can be present (not that it says large, not giant platelets in the stem)
154
A 6y old girl has diffuse aching in her arms, legs and back for more than 2 weeks. Results of laboratory tests include hemoglobin 94g/dl, wbc 5600 with no abnormal cells noted on smear and platelet count 106000. Radiographs of bone reveal osteolytic lesions and radiolucent metaphyseal growth arrest lines. Of the following the most likely cause of these findings :
a. ALL
b. Aplastic anemia
c. Gaucher disease
d. multifocal osteomylitis
gaucher
Gaucher disease is a lysosomal storage disease (one of the most common). Prevalent in Ashkenazi Jews. 3 types based on presence and progression of neurologic manifestations. Multisystem disease including:
Visceral: hepatomegaly, marked splenomegaly
Bone marrow disease: anemia and thrombocytopenia (leukopenia is rare)
Skeletal: diffuse bone pain punctuated by painful crises that often result in areas of AVN; osteolytic lesions, pathologic fractures and vertebral compression fractures occur from osteopenia
Growth + development: poor growth, delayed development
Imaging studies
Xray: Erlenmeyer flask deformity of distal femur, osteolytic bone lesions, pathological fractures
MRI: bone marrow infiltration, osteonecrosis
DEXA: osteopenia
155
A 3y old child has a somewhat unsteady gait, regression of language and is irritable. PE: mild left facial weakness, brisk reflexes, +ve babiniski and mild hypertonicity of the left upper and lower extremities. Most likely diagnosis:
a. Cerebellar astrocytoma
b. Pontine glioma
c. Post infectious encephalitis
d. tumor of the right cerebral hemisphere
b. Pontine glioma
156
Which of the following is most likely a sign of neutropenia?
a. disseminated herpes zoster
b. mucosal ulceration
c. subcutaneous nodules
d. urticaria
b. mucosal ulceration
157
Which of the following is present in tumour lysis syndrome:
a. hyperuricemia
b. hyponatremia
c. hypokalemia
d. hypophosphatemia
e. Hypercalcemia
a. hyperuricemia
158
All are features of iron deficiency anemia EXCEPT:
a. pica
b. Koilonychia (spoon nails)
c. cheilosis
d. mild scleral icterus
e. psychomotor retardation
mild scleral icterus
159
Which of the following is most likely to be seen in a child with Thalassemia minor?
a. Hgb 100, MCV 75, RBC 4.25
b. Hgb 100, MCV 60, RBC 4.81
c. Hgb 60, MCV 75, RBC 2.5
d. Hgb 60, MCV 60, RBC 2.5
b. Hgb 100, MCV 60, RBC 4.81
160
What is the most common cause of febrile reactions in blood transfusions?
a. sensitization to WBC antigens
b. hemolysis
2 types of acute transfusion reactions can present with fever
Febrile non-hemolytic reaction (1/300)
Acute hemolytic reaction (1/6000)
FNHTRs are common; these reactions are characterized by fever, usually accompanied by chills, in the absence of other systemic symptoms
The most common cause of FNHTR is release of cytokines from white blood cells (WBCs) in a product that has not been leukoreduced
Another cause is recipient antibodies, reactive to antigens expressed on cells in the transfusion, usually WBC
161
. In ITP:
a. you will find a big spleen
b. no proven link to preceding viral infection
c. CNS intracranial bleed is the worse complication
c. CNS intracranial bleed is the worse complication
162
A 3 year old girl with fever, arthralgia and lethargy for 10 days has lymphadenopathy, moderate hepatosplenomegaly, no obvious arthritis but screams in pain with minimal examination. WBC 9.5 Hgb 98 Plts 140, smear Normal. Next test:
a. bone marrow aspirate
b. EBV titers
c. follow
BMA
163
6 yo girl head tilt, diplopia, bilat papilledema, ataxia
a. craniopharyngioma
b. Dandy Walker cyst
c. brainstem glioma
d. cerebellar astrocytoma
d. cerebellar astrocytoma
Dandy-Walker malformation consists of a large posterior fossa cyst that is continuous with the fourth ventricle and defective development of the cerebellum, including partial or complete absence of the vermis. Hydrocephalus develops in 70 to 90 percent of patients
164
A 3-year-old girl has had persistent fever and sore throat for several days despite antibiotic therapy. There is ulceration of her tonsils and oropharynx, generalized lymphadenopathy, splenomegaly, and purpuric lesions on her lower extremities. WBC 25.6, hemoglobin 80, platelets 30. Most likely:
a. infectious mononucleosis
b. acute lymphocytic leukemia
c. idiopathic thrombocytopenic purpura
d. aplastic anemia
e. Lymphoma
ALL
EBV: purpura uncommon, usually only cervical lymphadenopathy
ITP: would not explain fever + leukocytosis, splenomegaly is rare, anemia is mild if at all
Aplastic anemia: need all 3 cell lines down
Lymphoma: HSM uncommon
165
An 8-year-old girl presents with fatigue. Blood work shows: hemoglobin 80, MCV 50, ferritin 150 mcg/L, serum iron 50 mmol/L. What is your next test:
a. barium meal
b. osmotic fragility test
c. abdominal Tc 99 scintigraphy
d. hemoglobin electrophoresis
e. bone marrow biopsy
d. hemoglobin electrophoresis
166
A 2-year-old is in your office for a routine check-up. His mother has no complaints. Growing well. You note pallor and do some blood work. Hemoglobin 80, low retic count. Smear is normocytic normochromic. Next investigation:
a. bone marrow aspirate
b. hemoglobin electrophoresis
c. Coombs' test
d. serum ferritin
BMA
167
Characteristics of Schwachman-Diamond syndrome
a. decreased pancreatic enzyme excretion
b. normal neutrophil count
c. hypocellular bone marrow
d. increased risk of diabetes mellitus
dec pancreatic enz excretion
168
A 16 year old teenage male has just received
chemotherapy for his leukemia. Now 48 hours
after administration, he has hematuria and mild dysuria but is otherwise well. His platelets were 90 before chemotherapy was given. What is the diagnosis?
a) Cyclophosphamide induced hemorrhagic cystitis
b) Myelosuppression with thrombocytopenia
c) Chlamydia urethritis
d) Urinary tract infection
a) Cyclophosphamide induced hemorrhagic cystitis
169
Teenage boy with stage III Hodgkin
lymphoma. Going to have chemo and
radiation.
List 2 factors that may affect his fertility
Give 2 options for having children in future
* Type of cumulative chemo and dose
* Radiation field (if near gonads)
* chemo - Azoospermia as a paraneoplastic phenomenon
* Sperm banking
* Adoption, donor sperm/IVF
170
3 side effect
vincristine
asparagainase
prednisone
vincristine- SIADH, peripheral neuropathy constipation, hyporeflexia, CNs)
- asparaginase: allergic reaction, heptopathy, coagulopathy/thrombosis, pancreatitis
prednisone: HTN, hyperglycemia
171
```
The most common organism isolated in episodes
of febrile neutropenia is?
a) PCP
b) E.Coli
c) CONS
d) Pseudomonas
e) Candida
```
CONS
usu give pip tazo which doesnt cover CONS but can add vanco
172
A 6-year-old boy with ALL completed a course of chemotherapy 1 month ago. He has a household contact with chickenpox, and he has never had it before.
Management?
a) isolate the patient and observe for symptoms
b) IM VZIG
c) IM VZIG + oral acyclovir
d) Admit + IV acyclovir
e) Admit + IM VZIG + IV acyclovir
VZIG only
173
Teen with cancer whose family now decides to
pursue palliative approaches. But his 10 yr old
brother, with whom he was quite close, starts
ignoring him, and spending not as much time with
him. His parents are concerned.
What do you tell them?
normal part, just greiving
