Heme/Onc - 2019 Updated! Flashcards
(176 cards)
1
Q
Scenario of a teen with cancer who family now decides to make him palliative. But his 10 yr old brother, with whom they were quite close as brothers, starts ignoring him, and spending not as much time with him. His parents are
concerned. What do you tell them? (1 point)
A
- it is normal for family members including siblings to experience anxiety, depression, guilt, fear and anger, which might make them pull away
- include in discussions about his brother’s care
- sibs who know about and are involved in the care of their dying sib cope better before and after their death
2
Q
Sickle cell girl who is obese, RUQ pain, increased liver enzymes, had been treated and recovers from the event. What do you recommend?
a. cholescystectomy
b. low-fat diet
c. ursodiol
A
a. Cholecystectomy
3
Q
- Child with Down syndrome. List three associated hematological disorders.
A
ALL AML Transient Myeloproliferative disorder Polycythemia Leukopenia Macrocytosis Thrombocytosis
4
Q
Left supraclavicular LN - mgmt?
a. Excisional biopsy
b. monitor
c. EBV serology
A
a. Excisional biopsy
5
Q
Management of Acute Chest Syndrome
A
- Supplemental Oxygen
- Blood Transfusion
- IV ABx: Cefotax and Azithro
- Pain managment
- Bronchodilators + steroids for concomitant asthma
- Incentive spirometry
6
Q
A girl presents with pallor and fatigue after a viral illness. Her laboratory findings are as follows: Hb 60, WBC 7, Platelets 250, Reticulocytes 359.
1) What is the most likely case of her anemia?
2) What 1 investigation will you do?
3) The blood bank calls to inform you that her plasma reacted to all tested cells. She has now clinically deteriorated and is hypotensive, tachycardic, and less responsive. What will you do with regards to transfusion?
A
1) Autoimmune hemolytic anemia
2) DAT
3) Transfuse O negative until you find our her blood group and DAT
7
Q
Newborn has a flank mass. What is the most likely cause:
Wilms tumour
Hydronephrosis
Polycystic kidney
A
Hydronephrosis
8
Q
Distinguishing features of congenital red cell aplasia (Diamond Blackfan Anemia) from Transient Erythobastopenia of Childhood
A
Both - Normochromic, Reticulocytopenia
TEC:
- Usually presents >12 mos (6mos-3years)
- Often follows a virus
- RBC adenosine deaminase (ADA) level is NORMAL
- Normocytic
DBA: - Presents early infancy (2-6mos) -Macrocytic - ADA is abnormal - Other features like craniofacial abs, thumb problems, GU anomalies, Cardiac (ASD, VSD, Coarct), Glaucoma, Cataracts, Short.
9
Q
A 6-year-old Chinese boy fell off his bike 24 hours ago. He is brought into the ER with right knee swelling and a large yellow bruise on his anterior chest wall. You worry about:
a) hemophilia
b) von Willebrand’s
c) thrombocytopenia
d) child abuse
e) herbal remedies
A
e) herbal remedies
Super bad ?racist question. But he’s a little old for a first presentation of hemophilia - but could also consider this as an answer.
A thorough history of medication use, including herbal medicines (eg, ginger, feverfew, ginkgo biloba, large amounts of garlic) is crucial. In particular, specific information should be sought about the ingestion of aspirin or NSAIDs.
10
Q
Which condition is most associated with childhood leukemia?
A. Electromagnetic field exposure during pregnancy
B. NF 1
C. Mom or dad with hx of leukemia
A
B. NF 1
11
Q
Standard Risk ALL
A
Age 1-10
Initial leuks <50
12
Q
Polycythemic newborn. Hb 240, Hct 0.75. Wt 2000g.
Child requires a partial exchange transfusion.
What fluid do you use as the diluent?
How much blood to you replace to decrease the Hct to 0.5?
A
- NS
- volume to be exchanged (ml) =
blood volume x (observed-desired hematocrit)/ observed hematocrit
- blood volume = 85ml/kg = 170ml in this case
- therefore tfn volume = 170 x (0.75-0.5)/0.75
= 56.6ml
13
Q
A VWD patient presents with bleeding. Which is the best treatment:
a. FFP
b. DDAVP
c. Cryoprecipitate
d. Platelets
A
b. DDAVP
Type 1 vWF (most common) - quantity issue - treated with desmopressin, increases amt of circulating VWF by releasing for storage.
Type 2 - quality issue - treated with vWF containing concentrate
Type 3 - severe quantity issue - treated with vWF containing concentrate
*cryo is best for low fibrinogen but can be used in a pinch for vWD, hemophilia A or B if specific factor concentrate is not available
14
Q
Description of newborn with petechiae. WBC normal, Hgb normal, plt 9. Rest of exam is normal. List 3 most likely causes aside from sepsis.
A
- neonatal alloimmune thrombocytopenia
- autoimmune thrombocytopenia (maternal ITP)
- TORCH infection (rubella, toxo, EBV, syphilis)
- Kassabach Merritt
- Renal Vein Thrombosis
- Indwelling catheter
- Type 2B vWB
- Hypersplenism
- Wiskott Aldrich
15
Q
3 yo with pallor, leg pain and fatigue for three weeks. Exam reveals tired, pale anicteric child with mild hepatosplenomegaly. No effusions or warmth/erythema
of legs. Most likely to confirm dx?
a. Abdominal ultrasound
b. Bone marrow aspirate
A
b. Bone marrow aspirate
Sounds like ALL
16
Q
28wk prem now 9wks of age. Mom breast feeding. Do they need iron supplementation and why?
A
Yes
Prems and LBW infants have smaller fetal iron stores and more rapid growth than term infants, anemia develops earlier
Start when BW doubled at ~4weeks (2-4mg/kg/day)
17
Q
A child with known hemophilia comes for routine immunizations. What would you advise with immunizations?
a. Delay them
b. give Factor VIII before immunizations
c apply pressure for 10 minutes
A
c. apply pressure for 10 minutes (and use small needle)
b. give Factor VIII before immunizations (if they are severe)
18
Q
You are counseling a mother and her young daughter with sickle cell anemia who requires a PRBC transfusion. Which of the following infections is she most at risk of acquiring from a transfusion:
a. Hep B
b. Hep C
c. Parvovirus B19
d. HIV
A
c. Parvovirus B19
- Hep B: 1 in 1.1-1.7 million
- Hep C: 1 in 5-7 million
- HIV: 1 in 8-12 million
- Parvovirus: 1 in 5000 to 1 in 20000
Most common transfusion related infection is yersinia enterocolitica
19
Q
All are features of iron deficiency anemia EXCEPT:
a) pica
b) koilonychia
c) cheilosis
d) mild scleral icterus
e) psychomotor retardation
A
d) mild scleral icterus
koilonychia are thin nails that lose their convexity - become spoon shaped.
20
Q
Which of the following would most likely be found in the bone marrow?
- Neuroblastoma
- Wilms tumour
- Hepatoblastoma
A
- Neuroblastoma
Most common sites of mets for Neuroblastoma - regional or distant LN, long bones and skull, bone marrow, liver and skin.
Wilms: lungs, LN and liver
Hepatoblastoma: regional LN and lungs
21
Q
You are seeing a pregnant woman during her first. Her father has haemophilia. Regarding the risk of her transmitting the disorder to her own children you tell her:
a. None will have it
b. 50% of her sons will have it and all of her daughters will be carriers
c. 50% of her sons will have it and 50% of her daughters will be carriers
A
c. 50% of her sons will have it and 50% of her daughters will be carriers
X-linked recessive inheritance
22
Q
A patient is being assessed for a bleeding diathesis. Labs show PT 12 s (normal), aPTT 50 s (prolonged), bleeding time 5 minutes (slightly prolonged), platelets 250,000.
a) hemophilia
b) von Willebrand’s disease
c) vitamin K deficiency
d) factor V deficiency
e) factor X deficiency
A
b) von Willebrand’s disease
or
a) Hemophilia
(depends if you think 5mins is long or not)
- vWD and hemophilia both have normal INR, long PTT, normal platelets, but vWD has normal or long bleeding time and hemophilia has normal bleeding time
- bleeding time is a bad test - should check vWF levels!
- vWF tethers platelets to damaged endothelium, and acts as a carrier protein for factor 8
23
Q
14 yo girl with leg pain, radiograph shows lesion in the distal femur with ‘sunburst’ appearance. What is the likely cause?
a. Osteoid osteoma
b. Ewing’s sarcoma
c. Osteogenic sarcoma
d. Aneurismal bone cyst
A
c. Osteogenic sarcoma (AKA osteosarcoma) - most common primary malignant bone tumour in children and teens. Classic sunburst pattern. Often metaphysical region of long bones, esp. knee.
- note: a. Osteoid osteoma (small benign bone tumour- unremitting, gradual increasing pain at night and relieved by aspirin; Tx- surgical removal - XR shows middle white spot surrounded by dense bone)
24
Q
An 8 year girl comes with the following skin finding [picture of large café-au-lait macule]. List 4 other skin findings in this condition.
A
NF1 skin findings:
- axillary or inguinal freckling
- neurofibromas
- Lisch nodules
25
What is the advantage of using leukoreduced white cells?
1. Decreases hemolytic reactions
2. Decreases infectious complications
3. Decreases febrile transfusion reactions
3. Decreases febrile transfusion reactions
The other's aren't really wrong either.
26
3 year old immigrated from West Africa. Appears well, has scleral icterus. Otherwise normal exam. Given CBC - normocytic anemia. Hg electrophoresis HgF50% HgC50% HgA 0%
a. What is the disease?
b. What are 2 complications
c. 2 indications for exchange transfusion in a patient with sickle cell?
a. Hemoglobin C disease
b. Hemolysis, Anemia
?stones
c. Acute emergencies: acute chest, stroke, splenic sequestration, stroke prevention
27
Kid with tumour lysis syndrome, what additional medication should he be on?
Rasburicase
give potassium
Rasburicase
Do NOT give K+
```
Hydration +++
Diuresis
Allopurinol
Rasburicase
Fix HyperK+ with kayexalate, Insulin+Glucose, Calcium-Gluconate
```
28
Infant (<1y) with distended abdomen and diffuse nonspecific skin lesions. Did have an abdominal ultrasound with a supra-renal mass. The urine was positive for HMA/VMA
a) What are your 3 non-renal differential for abdominal mass in a baby?
b) What additional investigations would you do?
c) What stage would you tell the parents?
d) What is the prognosis?
a) Neuroblastoma, hepatoblastoma, Teratoma, Leukemia (hepatomegaly)
b. Abdominal imaging (CT or MRI)
Tissue Bx or BM
MIBG (to assess extent)
CBCd, serum chemistries, liver and kidney function, LDH
c. 4S: Neuroblastoma in Infants <1 year with dissemination to liver, skin and/or bone marrow without bone involvement
d. Favorable, some regress without treatment
29
Child with hemihypertrophy.
What condition would you suspect that would require serial follow-up?
Beckwith-Wiedemann
Risk of abdominal tumors including Wilms and hepatoblastoma (get screening q3mos until 8) and neuroblastoma
30
How to treat macrophage activating syndrome?
High dose IV methylprednisone
Cyclosporine
or Anakinra
31
You are working in an Emergency department and a mother brings in a 2 year old girl with a petechial rash noted that morning while changing her diaper.
She is otherwise well-appearing. You suspect a diagnosis of ITP.
What are 4 indications for bone marrow aspiration in the setting of a probable diagnosis of ITP?
Basically anything that makes you think maybe it's actually leukaemia, not ITP
```
History
Constitutional symptoms *
Bone pain *
Recurrent thrombocytopenia
Poor treatment response
Physical
Generalised LAD *
Hepatomegaly *
Splenomegaly *
Unwell appearing child
Signs of chronic disease
Investigations
Anemia * (reminder that a mild anemia is allowable)
High MCV *
Abnormal WBC/neutrophil count *
Abnormal cellular morphology on smear *
```
32
Name four lab findings in tumor lysis syndrome
Hyperkalemia
Hyperuricemia
Hyperphosphatemia
Hypocalcemia
33
Late Hemorrhagic disease. What is most likely cause?
Breastfeeding
CF
Phenytoin
CF
Late onset > 2 weeks (up to 6 mos) is often chronic malabsorption.
34
What is the number one cause of complications in beta thalassemia major.
a. Iron overload
b. Megaloblastic anemia
c. Cardiomyopathy
d. Poor hematopoeisis
a. Iron overload
35
You are consulted on the postpartum ward for a neonate with thrombocytopenia. A CBC reveals a platelet count of 12. Following a platelet transfusion, the platelet count is 14. The baby’s mother has a normal CBC and a platelet count of 250. The baby has a normal newborn exam.
What is the most likely diagnosis?
a) Neonatal alloimmune thrombocytopenia
b) Neonatal autoimmune thrombocytopenia
c) Neonatal lupus
d) Sepsis
a) Neonatal alloimmune thrombocytopenia
36
Which is the most permanent toxicity of vincristine?
peripheral neuropathy
Seizures
Constipation
Peripheral neuropathy
37
Cyclosporine at start of treatment and high levels 3d into treatment. Pt started on clarithromycin. Reason?
1. renal transplant
2. clarithro inhibits renal excretion of cyclosporine
3. better compliance
4. Clarithromycin something else
Clarithromycin is a CYP3A Inhibitor
```
Other CYP3A Inhibitors:
Clairthromycin
Erythromycin
Itraconazole
Ketoconazole
Ritonvir
Diltiazem
Verapamil
Grapefruit
```
CYP3A help metabolize most drugs in the liver
38
A 10 year old boy with sickle cell anemia has multiple episodes of acute chest crises. Which of the following complications does this place him at increased risk for:
A. Stroke
B. Gall stones
C. Nephropathy
D. Asthma
A. Stroke
39
Baby born at home at 38 wks by midwife. Now presents at 7 days with melena. Hb 70, MCV 112, plts normal. What is the most likely diagnosis?
Vitamin K deficiency leading to hemorrhagic disease of the newborn
40
Newborn with platelets of 12, transfused and post-transfusion platelets were 16. Mom’s CBC was normal. What do you do?
a. transfuse single donor platelets
b. transfuse PLA-1 negative platelets
c. give IVIG
d. give steroids
b. transfuse PLA-1 negative platelets (AKA HPA-1)
- Treatment
o IVIG prenatally to mom
o C/S for delivery
o Transfusion
1. washed maternal platelets
2. cross-matched to mom’s serum
3. PLA-1 negative platelets
4. Random platelets (unlikely to improve as 98% of population PLA1 positive)
41
List 3 common causes of neutropenia .
- viral suppression
- chronic idiopathic neutropenia
- cyclic neutropenia
- drug induced (penicillins)
- leukemia/lymphoma
42
Older child who is at home for palliative care for her AML. (4)
What are four principles of treatment with opiods that would guide your treatment
```
WHO:
By the mouth (oral most convenient)
By the clock ( regular schedule)
By the ladder (stepwise approach)
Individualized to the child
```
- dosing needs to be individualized and titrated for the patient
- the right dose is one that relieves pain and minimizes side effects
- side effects should be anticipated and treated
- ensure good basal level of pain control with prn medication available for breakthrough pain
- administer medication via the simplest, most effective and least distressing route
43
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. Thymoma
B. Hodgkin’s lymphoma
C. ALL
D. Sarcoidosis
B. Hodgkin’s lymphoma
Hodgkin’s lymphoma is the most common malignancy in adolescence, though T-cell ALL may also have mediastinal mass.
Sarcoid more classicaly with bilateral hilar adenopathy
44
HB high at birth and now still at 3 months? most likely condition…
Down Syndrome
twin- twin transfusion
premature baby
Down Syndrome
Physiologic nadir is 6-8 weeks, if still high at 3 mos I'd think of something more chronic
Erythrocyte transfusion :
Delayed cord clamping, Intrapartum hypoxia (increased placental transfusion), twin-twin transfusion
Increased Intrauterine Erythropoiesis:
Placental insufficiency and chronic intrauterine hypoxia (SGA, Pre-Eclamplsia, Moms with cardiac or pulmonary disorders, smoking, high altitude)
IDM
LGA - includes Beckwith-Wiedemann
Endocrine Problems - CAH, hypo/hyper thyroid
T21, 19, 13
45
Newborn with T21 and petechiae, HSM. CBC showed elevated WBC, anemia, thrombocytopenia. What is the most likely diagnosis?
a) ALL
b) Fanconi anemia
c) transient myeloproliferative syndrome
d) sepsis
c) transient myeloproliferative syndrome
Approx. 10% of neonates with Down Syndrome develop a transient leukemia or myeloproliferative disorder → high leukocyte counts, blast cells in peripheral blood, associated anemia, thrombocytopenia and HSM
Features usually resolve within first 3 months of life
20-30% who develop TMD will develop leukemia by age 3
46
Child macrocytic anemia, bifid thumbs. What else to expect on exam:
craniofacial anomalies
absent radii
Craniofacial anomalies
Diamond Blackfan Anemia (DBA)
- craniofacial (hypertelorism, high arch palate, cleft palates, microcephalic, micrognathia, low ears, low hairline)
- eyes (congenital glaucoma, strabismus, congenital cataract)
- neck (short, webbed)
- thumbs (triphalangeal, duplex or bifid, hypoplastic, absent radial artery)
- GU (hypospadias, absent kidney, horseshoe) - Cardiac (Vsd, ASD, coarct)
- growth retardation - learning problem
47
Hereditary Spherocytosis:
a) What would you use to diagnose this
b) What would be indications for splenectomy
a)
Can Establish with:
- Positive family history
- Lab/PE Findings:
Splenomegaly, spherocytes on blood smear, reticulocytosis, elevated mean corpuscular hemoglobin concentration
If uncertain:
- Flow cytometry EMA (eosin-5-maleimide) binding test
- osmotic fragility
b) Severe HS: frequent hypo plastic or aplastic crisis, poor growth, cardiomegaly.
48
Teen on OCP swelling of leg in ED develops chest pain/shortness of breath. Management:
1. Coumadin
2. Heparin
3. TPA
4. Surgical removal of clot
2. Heparin (or LMWH)
Coumadin - longer term after acute episode
TPA: Use with anticoagulants in hemodynamically significant PE
Surgery - limited to persistent hemodynamic compromise
49
Long term consequence of fanconi anemia?
- pancytopenia - bone marrow failure
- AML
- squamous cell carcinoma of head, neck and esophagus
50
Boy with fever, wt loss, splenomegaly, fatigue
a) CT abdo
b) CXR
b) CXR
Splenomegaly Initial diagnostic testing
```
Complete blood count (CBCd)
Reticulocyte count
Review of the peripheral blood smear
Liver function tests
EBV and CMV serologies
Chest radiograph
Abdominal ultrasound
```
51
Teenager has lymphadenopathy (including supraclavicular) and fevers, weight loss. What do you do?
a. abdominal ultrasound
b. chest xray
c. CT chest/abdo
d. bone marrow
b. chest xray
Look for mediastinal mass - lymphoma
52
```
A child’s newborn screen was positive, with the following pattern:
HbA – None,
HbF – 70%,
HbS – 20%,
HbA2 – 10%.
```
What is your diagnosis:
a) alpha thalsemia
b) sickle cell trait
c) beta thalasemmia
d) sickle cell disease
d) sickle cell disease
Has NO HbA
FAS = sickle cell trait (HbA > HbS)
FSA = Sickle beta thal (HbS >HbA)
Hemoglobin F: alpha2 gamma2
Hemoglobin A : alpha2 beta2
Hemoglobin A2: alpha2 delta2
Hemoglobin S: alpha2 betaS2 (abnormal beta S globin )
53
In which of the following situations should you investigate a child with sickle cell anemia for possible stroke:
A. Increasing lethargy
B. Recent poor school performance
C. Acute worsening of anemia
B. Recent poor school performance
- silent cerebral infarct common in kids with sickle cell
** increasing lethargy would be a CRITICAL sign though?
54
A child with thalassemia major on recurrent transfusions and desfuroxime. What is the most serious complication:
a. cardiac hemosiderosis
b. chronic anemia
c. cognitive impairment
d. liver hemosiderosis
a. cardiac hemosiderosis
- 1 year of tfns = liver hemosiderosis
- 2 years of tfns = endo (hypopara, hypogonadotropic hypogonadism)
- 10 years = cardiac - this is what kills you if you don't have iron chelation therapy
55
Which of the following is inherited in an autosomal dominant fashion?
1) G6PD
2) Hereditary Spherocytosis
2) Hereditary Spherocytosis
HS: Most common AD, spectrum of asymptomatic to severe hemolytic anemia
G6PD: X-linked. Presents as neonatal jaundice or chronic intermittent jaundice triggered by infection, drugs, fava beans.
56
Child with microcytic hypochromic anemia. You believe it is Fe deficiency.
What other features of CBC would be consistent with this? List 3.
- increased RDW
- RBC count decreased
- MCH decreased
- WBC count normal
- thrombocytosis
- low retics
57
Reason why you can’t get therapeutic heparin for oncology kid with clot:
Antithrombin III Deficiency
Factor V Leiden
Protein C Deficiency
Antithrombin III Deficiency
UFH and LMWH act by catalyzing action of antithrombin.
Asparaginase (used in ALL) reduced AT
58
What treatment is proven to prevent stroke in SCD?
a. transfusion
b. folate
c. hydroxyurea
d. Iron
a. transfusion
Based on monitoring transcranial dopplers
59
```
1 mo baby with FTT and diarrhea.
WBC 5 x109 :
80% neutrophils
10% lymphocytes
6% basophils
4% eosinophils
```
a. ) What does this make you think of? (i.e. interpret the labs)
b. ) List 2 causes:
a)
10% Lymphocytes = 0.5 (low)
Definition of lymphopenia varies according to age
<12 mo: lymph <3
> 12 mo: lymph < 1
b) SCID, DiGeorge, protein losing enteropathy, Wiskott-Aldrich, virus (HIV)
60
The severity of Rh incompatibility at the time of birth is best predicted by:
a. cord Hb
b. cord bilirubin
c. prematurity
d. splenomegaly
e. maternal anti-D antibody
a. cord Hb
Weekly monitoring is performed with the antibody titer and ultrasonography. Ultrasound is performed for MCA peak systolic velocity (MCA-PSV) and for any evidence of fetal hydrop.
Severity related to level of anemia and degree of reduction in serum albumin.
61
List 4 indications for blood transfusion in sickle cell disease
Treatment of acute complications:
- acute chest syndrome
- aplastic crisis
- stroke
- splenic sequestration (but be careful with this due to auto transfusion)
Prevention:
- prevent stroke in pt with abnormal intracranial dopplers
- prior to surgery to prevent ACS after
62
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?
1) Limit milk to 500cc/day
2) Start elemental iron 60mg PO tid
3) Give PRBC transfusion 130cc
4) Change to protein hydrosylate formula
a) Limit milk
You'd also treat him but treatment dose would be less than what they've written.
Not symptomatic for transfusion (and I'd probably Tx Iron over pRBCs)
63
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
a) bone marrow aspirate
Due to bicytopenia and systemic features.
EBV - rarely apparent in kids <4 but in older kids has the classic triad of : fatigue, pharyngitis, generalized lymphadenopathy.
64
Neonatal hemophilia – 4 clinical presentation in the neonatal period
- factor VIII and IX do not cross the placenta so symptoms can be present in utero or from birth
- extracranial hemorrhage (cephalohematoma, subgaleal)
- intracranial hemorrhage (subarachnoid, smbgaleal)
- prolonged bleeding with circumcision (30%)
- prolonged bleeding from heel poke/venipuncture (newborn screen)
65
ALL kid just had chemo. He is sexually active. Now 48 h after has hematuria and mild dysuria. Otherwise well. Plts were 90 before chemo. Dx?
A. Cyclophosphamide induced hemorrhagic cystitis
B. Myelosuppression with thrombocytopenia
C. Chlamydia urethritis
D. UTI
A. Cyclophosphamide induced hemorrhagic cystitis
Usually see 24-48h after chemo and lasts 4-5 days.
Prevention: hydration and mesna
66
Child with hemihypertrophy. Other than Wilms tumors, what other neoplasm do you need to screen for:
a) Neuroblastoma
b) Hepatoblastoma
c) No other neoplasms
b) Hepatoblastoma
- Neuroblastoma screening has not been incorporated into most screening protocols
Beckwith-Wiedemann:
Risk of Wilms tumor, hepatoblastoma, neuroblastoma, rhabdomyosarcoma
A/W macrosomia, macroglossia, hemihypertrophy, omphalocele
67
12 year old M with recent change in behaviour, 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
A. Brain Tumour
68
4 year old of Mediterranean descent. Lab findings most consistent with Thal 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 80 MCV 60 RBC 3.3
e. Hb 60 NCV 50 RBC 2.2
ANSWER: a. Hb 100 MCV 60 RBC 4.8 (Mentzer= 12.5)
Alpha or beta thal= Should see: microcytic anemia with high RBC count and low or normal retic
- Mentzer Index: MCV/ RBC count in millions
o > 13= iron deficiency more common; <13 beta thal more likely
69
Labs in keeping with hemophilia? and effect of mixing studies?
Prolonged PTT - 2-3 x ULN
- when you mix normal plasma with patient plasma the PTT corrects
- platelets and INR are normal (in the lab, INR measures activation of factor X directly by factor VII, BUT in real life factor VII activates factor IX which activates factor X so there is a real life deficiency, but it's not picked up in the lab)
- specific assays for factor VIII and Ix show deficiency
70
List 3 treatments for hyperuricemia
- allopurinol (blocks enzyme in pathway that makes uric acid)
- rasburicase (makes uric acid more water soluble and easily excreted)
- dialysis
- IVF
- Treat electrolyte disturbance (will also have hyper K in tumor lysis)
(Alt Q: tumor lysis bloodwork, what do you give?)
71
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
b) acute lymphocytic leukemia
2 cell lines low, increased WBC, splenomegaly, generalized lymphadenopathy
IM - shouldn't give anemia and wrong age for this presentation
ITP - should be just PLTs
AA - all line should be down
Lymphoma - wrong age
72
18 mos with Leukocoria.
What is the most important Dx to consider? Name two others.
Retinoblastoma
```
Cataracts
Stage 4-5 ROP
Coats Disease
Traumatic retinal detachment
Posterior coloboma
```
73
8 year old healthy child with 1.5 x 2 cm supraclavicular lymph node, firm, non-tender, mobile with no erythema. What to do?
a) Excisional biopsy
b) PPD (TB test)
c) Bartonella serology
d) EBV serology
a) Excisional biopsy
74
Child with sickle cell. Admitted for Dactylitis in the past. Biggest risk factor for poor prognosis:
Male sex
Previous admission for Dactylitis
Baseline HGB
Baseline Hgb
Poor prognostic factors:
Dactylitis <1 year
Low baseline Hgb
High leukocytes w/o infection
75
What is the most common cause of febrile reactions in blood transfusions?
a) sensitization to WBC antigens
b) hemolysis
d) Cytokines produced and released in the storage of blood products
d) Cytokines produced and released in the storage of blood products
A is also true - but this is more true.
76
Infant 2 yold with recent viral illness, CBC shows WBC 5, Plt high normal, see normocytic anemia with reticulocyte count 0
1. Transient erythroblastopenia of childhood
2. Diamond Blackfan
3. Leukemia
4. Iron deficiency anemia
1.Transient erythroblastopenia of childhood → right age group, after viral illness
Diamond Blackfan → anemia (normochromic and macrocytic), reticulocytopenia and insufficient/absent RBC precursors; would present at earlier age and have abnormal clinical features
Leukemia → would present with low plts, low hgb and high WBC
Iron deficiency anemia → microcytic anemia
77
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
c. Stop transfusion, run IV TKVO
Febrile non-hemolytic transfusion reaction - stop the transfusion, give antipyretics and monitor
If fever 38-39C, limited symptoms can consider acetaminophen, then can restart transfusion cautiously
Do not restart transfusion if any other systemic symptoms than fever, chills
78
Paraneoplastic syndrome associated with neuroblastoma:
a) chorea
b) athetosis
c) diarrhea
d) hypertension
e) hypercalcemia
c) diarrhea
- due to secretion of vasointestinal peptide (VIP), leading to hypokalemia and dehydration
Opsoclonus-myoclonus-ataxia syndrome is most common
79
Name 3 syndromes associated with increased risk of leukemia:
T21
Tumor Suppressor:
- NF1
- Li-Fraumeni
Chromosomal Breakage:
- Fanconi anemia
- Bloom syndrome
- Ataxia telangiectasia
Bone Marrow Failure:
- Schwachman Diamond
- Kostmann Syndrome
- Diamond-Blackfan
- Thrombocytopenia Absent Radius
Immunodeficiency:
Wiscott-Aldrich
X-linked Agammaglobulinemia
80
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
D. Fever > 39
A. ANC <1000
(most concerning)
*C. You are allowed to have mild anemia due to blood loss.
In classic ITP you don't need to do a BM Bx. Only do if you need to rule out other causes
- Malignant features : LN enlargement, splenomegaly, bone/jt pain, B symptoms, Neutropenia, leukocytosis, atypical lymphocytes or MARKED anemia
- Insufficient or no response to steroids, IVIG, or Anti-D
81
A woman is a carrier of hemophilia A. Her husband is a haemophiliac, type A. The couple is expecting a baby girl. What is her risk of being haemophiliac?
a) 25%
b) 33%
c) 50%
d) 100%
c) 50%
→ since baby is a girl, dad will pass on affected X chromosome; then there is 50% chance mom will pass on affected X chromosome to baby (since mom is a carrier)
82
List 6 longterm side effects of Hodkin's Lymphoma treatment - radiation to neck and chest, chemo of cyclophosphamide, VCR plus others.
```
Radiation treatment:
Growth retardation
Thyrioid dysfunction
Cardiac toxicity
Pulmonary toxicity
Secondary cancers: brain tumours, breast cancer, thyroud cancer, lung cancer, upper GI cancers
Neurocognitive impairment
Hering impairment
Vision impairment/cataracts
GH deficiency
Hypothalamic hypogonadism
Dental anomalies
Esophageal strictures
Lung fibrosis
```
Chemo - Long Term
Bleomycin : pulmonary fibrosis
Cyclophos (alkylating): gonadal dysfunction, infertility
Vincristine: peripheral neuropathy
Doxorubicin : dilated cardiomyopathy
Methotrexate intrathecal : neurocognitive impairment
Cisplatin: renal disease
83
3 week old who is brought to the office because mother thinks he is too yellow. Breastfed. Otherwise well. Total bili is 180. Direct is 8. What do you do?
A. septic workup
B. investigate for blood group incompatibility
C. reassure mother that condition may last for 4-12 weeks
D. investigate for metabolic disease
C. reassure mother that condition may last for 4-12 weeks
Breast milk jaundice
84
List 3 side effects of Prednisone
```
H&N:
Cataracts, glaucoma
Cervical fat pad
Moon faces
CNS
Psychosis
Emotional lability
Idiopathic intracranial HTN
Sleep disturbance
CVS
HTN
GI
Peptic ulcers
Gastritis
Increased appetite
Weight gain
GU
Sodium retention
Fluid overload
Potassium loss
Decreased sperm count
Endo
Cushing syndrome
Diabetes
Irregular menses
Adrenal suppression
Growth suppression
MSK
AVN
Myopathy
Osteoporosis
Pathological fractures
Derm
Striae
Acne
Impaired wound healing
Thin hair
Hirsutism
ID
Infection
Immunosuppression
```
85
Child comes to you with headache list 5 features on history that would make you think this is a brain tumor (Specifically a infratentorial brain tumor)
Wakes from sleep
Associated with vomiting
AM headache that improves through the day
Associated with diploplia
Ataxia
Cranial nerve dysfunction (facial weakness, swallow dysfunction, oculomotor abn)
86
Complication of doxorubicin?
Restrictive cardiomyopathy
Dilated cardiomyopathy
Hypertrophyic cardiomyopathy
Pericarditis
Dilated cardiomyopathy
87
What would be the Hb/MCV/RBC findings in a child with thalassemia minor
Mild microcytic anemia with normal/elevated RBC
Mentzer < 13
88
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, retic count 1%, MCV 85. Smear is normocytic normochromic. Next investigation:
a) bone marrow aspirate
b) hemoglobin electrophoresis
c) Coombs' test
d) serum ferritin
e) RBC osmotic fragility test
a) bone marrow aspirate
BW does not fit with thalassemia (microcytic), sickle cell (high retics), hemolysis (high retics) or iron deficiency (microcytic) for BMA best choice by elimination.
*IF there was an option to do nothing and repeat in one month, would consider that as could be TEC
89
Kid on methotrexate for systemic JIA. Comes in with irritability, abdo pain, petechiae, hepatomegaly. Elevated liver enzymes, ESR 30. No CBC.
What’s her most likely diagnosis?
a. methotrexate toxicity
b. sepsis
c. autoimmune hepatitis
d. macrophage activation syndrome
d. MAS
Rare but potentially fatal complication of sJIA that can occur at any time
Classically manifests as: acute onset of high spiking fevers, lymphadenopathy, HSM, encephalopathy; may have purpura and mucosal bleeding
90
```
3 yo Sickle cell, with fever cough, tachypnea, unwell. What do you need to rule out 1 st ?
A. Acute chest crisis
B. PE
C. Pneumonia
D. Asthma
```
A. Acute chest crisis
Definition:
- New radio density of CXR
- Plus 2 of: fever, respiratory distress, hypoxia, cough, chest pain
91
What is the most common complication of hereditary spherocytosis:
a. aplastic anemia
b. gallstones
c. splenic infarcts
d. frontal bossing
ANSWER: b. gallstones (most common) due to chronic hemolysis
a. aplastic anemia (at risk if get parvoivrus B19 - have high turnover of RBCs so if marrow production is affected they're in trouble)
d. frontal bossing (can get but more common in thal major (extra medullary hematopoiesis))
92
List one associated malignancy for:
Aniridia
Beckwith-Wiedemann
Trisomy 21
Aniridia: Wilms (WAGR)
Beckwith-Wiedemann: Wilms, Hepatoblastoma, Neuroblastoma
Trisomy 21: Leukemia
NF1: Leukemia, ++
93
Teen with 2.5cm supraclavicular LN. 2kg weight loss, nightweats. Management?
Pip Tazo
IV fluids, allopurinol
IV steroids
IVF and allopurinol
To reduce risk of tumor lysis syndrome:
Happens in patients with large tumor burden (burkitt lymphoma, lymphoblastic lymphoma, high WBC leukemia) - usually seen in 12-48h of starting chemo
Before starting treatment measure serum uric acid, electrolytes, calcium, phosphorus and creatinine. Ensure adequate hydration. Allopurinol should to started to prevent further accumulation of uric acid
94
What long term complication is associated with the following chemotherapy drugs
```
Cisplatin :
Bleomycin :
Cyclophosphamide :
Donorubicin (as in Daunorubicin or Doxorubicin) :
Vincristine :
```
Cisplatin : Sensorineural Hearing Loss, Peripheral Sensory Neuropathy
Bleomycin : Pulmonary Fibrosis
Cyclophosphamide : Infertility
Donorubicin (as in Daunorubicin or Doxorubicin) : Cardiomyopathy
Vincristine : Peripheral motor and sensory neuropathy
95
Poor prognosis for Langerhans cell histiocytosis is
associated with:
a) mastoiditis
b) pancytopenia
c) lymphadenopathy
d) chronic lung disease
e) vertebral body involvement
b) pancytopenia
Risk organs: liver, spleen and bone marrow (denote a worse prognosis). Mastoiditis presents a RF for CNS involvement.
96
A child is admitted to the pediatric intensive care unit for cerebral hemorrhage. The medical student working with you performs a thorough history, and notes a family history of Von Willebrand disease type III.
Knowing this, what will you prescribe?
a) DDAVP IV
b) Cryoprecipitate
c) Fresh frozen plasma
d) von Willebrand and factor VIII concentrate
d) von Willebrand and factor VIII concentrate
Type III vWB - severe qualitative defects that presents similar to hemophilia with mucosal + jt bleeds + CNS hemorrhage.
97
6 month old baby with pallor, hepatosplenomegaly, irritable. Xray shown with very white bones. Diagnosis:
A. osteopetrosis
B. osteoid osteoma
C. rickets
A. osteopetrosis
(infantile malignent osteopetrosis)
- increased bone density leads to marrow replacement - pancytopenia
98
Baby with ambiguous genitalia, proteinuria, and edema. What disease is this baby at risk of developing?
Wilms tumour
Adrenocortical carcinoma
Congenital mesoblastic nephroma
Neuroblastoma
1. Wilms Tumor
Denys-Drash Syndrome -
Nephropathy, ambiguous genitalia, bilateral aims tumor
99
Child with Kawasaki. Treated with IVIG and ASA. Develops gross hematuria, 24 hours after IVIG. Urinalysis shows 3+ Blood/Protein. Did not give microscopy. Elevated non-conguted bili. Elevated transaminases, elevated LDH. What is the most likely etiology?
Thrombosis of renal vein
Hemolysis from IVIG
Nephritis from Kawasaki disease
Hemolysis from IVIG
- alloantibodies can cause immune hemolysis, usually self limited with reduced haptoglobin, increased LDH and transient reticulocytosis
100
Fullterm baby delivered to an O+ mom. Looks well but pale. Hb is 70, he is hemodynamically stable. What is the most likely diagnosis?
1. ABO incompatibility
2. Chronic fetal maternal hemorrhage
3. Rh incompatibility
2. Chronic fetal maternal hemorrhage
ABO incompatibility: no pallor at birth; presents with jaundice in first 24 hours
101
Teen with stage 4 lymphoma and vomiting/hyponatremia. Side effect of which med?
Vincristine
Cyclophosphamide
Prednisone
Adriamycin
Cyclophosphamide
SE: nausea, vomiting, hemorrhagic cystitis, pulmonary fibrosis, SIADH,
Vincristine: peripheral neuropathy, constipation, ileum, jaw pain, SIADH, seizures
Prednisone: Cushing syndrome, cataracts, diabets, HTN, myopathy, osteoporosis, AVN, infection, peptic ulcer, etc.
Adriamycin (Doxorubicin): N/V, cardiomyopathy, red urine, tissue necrosis, conjunctivitis, arrhythmia, etc.
102
Wilm’s tumour is associated with all of the following except:
a. 11p abnormalities
b. NF
c. Wiskott-Aldrich
d. Aniridia
e. Beckwith Wiedemann
c. Wiskott-Aldrich (is a RF for leukaemia and lymphoma)
NF1 rare/contraversial as well
- 11p abnormalities, even without BES, can be associated with increased risk of WIlm's tumour (nephroblastoma)
- WAGR (Wilms, aniridia, GU anomalies, mR)
103
What are the 3 investigations for hereditary thrombosis?
```
Factor V Leiden
Prothrombin mutation
Antithrombin III deficiency
Protein S
Protein C
Elevated Factor VIII
Homocystinuria
```
104
Child with inguinal adenopathy found 1 week ago by parent while bathing.
Give 4 indications for biopsy.
B Symptoms ; fevers, weight loss, night sweats
Hard and fixed nodes, non-tender
Other LN including supraclavicular nodes
Rapid increase in size in past 2 weeks, no decrease in 2-4 weeks
Abdominal Mass
Abnormal CXR
Abnormal CBC+diff
105
irradiated PRBCs when given to neonates to :
1. avoid GVHD
2. avoid hemolytic anemia
3. make blood sterile
4. eradicate CMV
1. avoid GVHD
106
Child with severe hemophilia. Mom gives factor VIII at home prophylactically, but she has been finding that recently he has not been responding as well. Today he has an ankle bleed. What do you do?
A) RICE the ankle
B) Check for inihibitors and do factor VIII levels
C) Give DDAVP
D) Switch to a different brand for factor VIII
B) Check for inihibitors and do factor VIII levels
Most serious iatrogenic complication of hemophilia is development of factor inhibitors.
DDAVP not helpful with severe hemophilia
107
A 6-year-old boy with acute lymphocytic leukemia 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
b) IM VZIG
Use within 96h, but can be useful up to 10 days.
Do not vaccinate (live vaccine)
If you don't know immune status then check Ig levels and give VZIG.
If kid is post stem cell transplant just give VZIG because they won't have any immunity anymore (if they ever had it)
If the develop symptoms then start antivirals.
108
A blood smear of a patient with iron deficiency anemia would show all EXCEPT:
a) Heinz bodies
b) poikilocytes
c) microcytosis
d) hypochromasia
e) normal or low reticulocyte count
a) Heinz bodies - see in G6PD or thal
Poikilocytes (abnormal shape cell as general term- yes can see elliptocytic or cigar shaped RBC)
109
Opsoclonus is seen in which of the following:
a. AML
b. ALL
c. Neuroblastoma
d. Medulloblastoma
e. Rhabdomyosarcoma
c. Neuroblastoma
Neuroblastoma can also be associated with a paraneoplastic syndrome of autoimmune origin, termed opsoclonus– myoclonus–ataxia syndrome, in which patients experience rapid, uncontrollable jerking eye and body movements, poor coordination, and cognitive dysfunction.
110
An 8-year-old girl presents with fatigue. Blood work shows: hemoglobin 80, MCV 50, ferritin 150 μ g/L, serum iron 50 μ mol/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
- microcytic anemia + normal iron studies = thalassemia
*osmotic fragility test is for hereditary spherocytosis
Mentzer index = MCV/RBC when <13 more likely thal
111
Classic timing for VitK deficient breast fed baby to bleed?
2-7 days
112
2 yo child with new onset petechiae and purpura, nosebleed yesterday. Remainder of physical exam normal. Had viral infection 1 week prior. WBC 5, Hb 100, Plt 5. Smear normal.
a. ) Most likely diagnosis.
b. ) List 2 modalities of treatment for this condition.
a. ITP
b. reassurance and watchful waiting is generally indicated
- if treating could use single dose of IVIG or short course of corticosteroids
113
Sickle cell anemia, which is true about strokes?
a . usually subclinical strokes
b. clinical stroke
a . usually subclinical strokes
114
A child presents with eczematous lesions on the diaper area, lymphadenopathy, cytopenias. On xray there are multiple lytic lesions on the skull. What is your diagnosis?
a) immunodeficiency
b) Leukemia
c) Langherhans cell histiocytosis
c) Langherhans cell histiocytosis
115
Mediastinal Mass- Do you do a fine needle aspirate?
HECK NO
Need anesthesia, ECHO, PFT
CT
116
7 year old with severe anemia. BM shows arrest of erythroid precursors. Dx?
a. Aplastic anemia
b. TEC
b. TEC
- platelets normal or elevated, retics low
- prognosis: spontaneous recovery in 1-2 months
* aplastic anemia: absence of hematopoietic stem cells
117
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
cerebella astrocytoma and medulloblastoma are most common posterior fossa tumours.
Brainstem glioma could also cause the diplopia, unsteadiness and weakness. and increased ICP. Account for 10-15%
118
Clinical and Laboratory Criteria of Macrophage activating Syndrome
```
Non remitting fever
Hepatomegaly
Splenomegaly
Lymphadenopathy
Hemorrhage
CNS Dysfunction
```
```
Lab:
Cytopenias
Abnormal LFTs
Coagulopathy (hypofibrinoginemia)
Decreased ESR
Hypertriglyceridemia
Hyponatremia
Hypoalbuminemia
Hyperferritinemia
High sCD25, sCD163
```
The fall in ESR is helpful to distinguish from flare of underlying Dx (like sJIA)
Histiopathologic: Macrophage hemophagocytosis in BMA, increased CD163 staining
119
Transient fever in blood transfusions is usually a result of:
a. sensitization to WBC antigens
b. acute GVHD
c. hepatitis C
d. hemolysis
a) sensitization to WBC antigens (leukoreduction helps prevent this)
- hemolytic reactions can cause fever but are not transient and not the the most common
120
Kid with pancytopenia, short stature, abnormal thumbs. What does he have?
Fanconi anemia
Hyperpigmentation of the trunk, neck, intertriginous areas, as well as cafe-au-lait spots and vitilgo
Short stature (50%)
Absence of radii and thumbs that are hypoplastic, supernumerary, bifid, or absent
The “r” radial pulse may be weak or absent
Anomalies of the feet, congenital hip dislocation, and leg abnormalities are seen
In males: underdeveloped penis, undescended, atrophic or absent testes, hypospadias or phimosis
Females: malformations of the vagina, uterus and ovaries
FA “facies”: microcephaly, small eyes, epicanthal folds, and abnormal shape, size or positioning of the ears
121
4 yo child with abd mass, looks pale, Hb 80, WBC 4.
List the Ddx (3).
List 3 investigations which will help you distinguish between the diseases listed in your differential before a biopsy can be done.
```
1. Ddx:
Solid Tumors:
- wilms tumour
- neuroblastoma
- hepatoblastoma
- teratoma
```
Heme Malignancy:
-lymphoma, leukemia
Organomegaly -
Hepatomegaly, splenomegaly
2. Ix:
Urine VMA (vanillylmandelic) and HVA (homovanillic) for neuroblastoma
- U/S
- CBC (polycythemia and thrombocytosis in Wilms) and peripheral blood smear
- U/A (hematuria with Wilms)
- increased alpha-fetoprotein, betaHCG for hepatoblastoma
- MIBG
122
Which of the following is present in tumour lysis syndrome:
a) hyperuricemia
b) hyponatremia
c) hypokalemia
d) hypophosphatemia
e) hypercalcemia
a) hyperuricemia
HyperPO4, hyperK, hypoCa
Ca is low because it gets bound by phosphate
123
High Risk ALL Patients
- Children less than 1 year and older than 10
- Initial leuks >50
- Bad immunophenotype or slow response to therapy
- Chromosomal abnormalities (hyPOploidy, philadelphia, MLL, etc)
124
Which of the following predisposes to early hemorrhagic disease of the newborn:
a) breastfeeding
b) prematurity
c) cystic fibrosis
d) maternal phenytoin (what we always used to say BUT recent evidence does
not support this but says clinical practice up to you (look at mother risk))
e) diarrhea
ANSWER: d) maternal phenytoin (now actually somewhat controversial in evidence, but best answer)
a) breastfeeding - at higher risk for late onset, especially if never got vitamin K
c) CF - used to think this was true (malabsorption), but evidence does not support this
125
A full term newborn develops petechiae and bruising. The baby is otherwise well appearing. On bloodwork his platelets are 12, WBCs are 18 and his Hgb is 140. He is
given a platelet transfusion and a repeat platelet count is 16. The mother’s CBC shows platelets of 80. What is the best treatment:
A. PLA-1 negative platelets
B. IVIG
C. Washed maternal platelets
ANSWER: b - IVIG
This is autoimmune (likely maternal ITP) - mom and baby have TCP
Treat with IVIG +/- Steroids
A. PLA-1 negative platelets → NAIT
B. IVIG → Can be used in NAIT (prenatal transfusion to mom) and AI but more effective in AI than NAIT
C. Washed maternal platelets → NAIT
126
30 month easy bruising with bruises on bony prominences and thorax, otherwise exam unremarkable thrombocytopenia 23 large platelets on smear, no blasts, hb 125, wbc 9.1.
What is the MOST appropriate management:
a. 1g/kg IVIG once with close follow
b. 2mg/kg prednisone X 4d with close follow up
c. Avoid high risk activity and NSAIDs and close follow up
d. Anti D IG
c. Avoid high risk activity and NSAIDS and have close follow-up
This sounds like ITP (large platelets in acute ITP due to increased turnover)
Observation if - mild bleeding and prompt follow-up available, no social or distance concerns, no excess activity, family anxiety low
Otherwise steroids are first line Tx
127
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
a. DIC work-up
Kasabach Merritt
DIC = Platelets low, INR (normal-high), PTT (normal-high), fibrinogen (low), D-dimer (high)
128
A child is brought in to see you with 3 days of high fever of 40.1 degrees and feeling unwell. The only thing you see on physical exam is clear rhinorrhea. A CBC shows the following:
Hb 118,
WBC 2.0x 10^9 (2000/m3), Platelets 250.
The differential shows neutrophils 2%, lymphocytes 70%, eosinophils 8%.
What are TWO things that you will do in the management of this child?
Febrile Neutropenia
1. Draw blood cultures
2. Start IV ABx (Pip-tazo)
3. Admit to hospital
129
6 year old who has a history of diplopia, headache and
| ataxia. Where is the lesion? (1) What are the two most likely brain tumours for the lesion (2)
1. posterior fossa (brainstem and cerebellum)
2. cerebellar astrocytoma and medulloblastoma
brainstem glioma is less likely
130
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
ITP is a problem of immune destruction, not splenic sequestration.
60% of kids have a preceding viral infection.
131
Most consistent with DIC:
a) increased fibrinogen
b) increased PTT
c) decreased PT
d) decreased INR
e) decreased fibrin split products
b) increased PTT
- high d-dimer (fibrin split products)
- low platelets
- prolonged prothrombin time
- low fibrinogen
o Blood replacement (PRBCs) for haemorrhage
▪ +/- plt (thrombocytopenia)
▪ +/- cryoprecipitate (for low fibrinogen)
▪ +/- FFP (replace coagulation factor + natural inhibitors)
132
Give four laboratory findings in tumor lysis syndrome.
- hyperkalemia
- hyperphosphatemia
- hypocalcemia
- hyeruricemia
133
A 12 year old adolescent presents to your clinic with chest pain. He has been febrile for several days and describes left lateral chest pain. You order a chest radiograph that reveals a mottled area within the 8th rib with a periosteal reaction. The most likely diagnosis is:
a) Ewing’s sarcoma
b) Osteomyelitis
c) Osteosarcoma
d) Costochondroma
Ewing sarcoma
134
What to avoid in G6PD
```
Fava Beans
Sulfa drugs
Antimalarial drugs (primaquine, chloroquine)
ASA
Rasburicase
```
X linked enzymatic deficiency, resulting in either episodic hemolytic anemia (induced by infections, certain drugs, fava beans!), or chronic nonspherocytic hemolytic anemia
+Heinz bodies
135
Newborn with non-hemorrhagic stroke – 4 inheritable etiologies for clot
- congenital heart disease
- factor V Leiden
- prothrombin gene mutation
- protein C/S deficiency
- antithrombin III deficiency
- antiphospholipid antibody (from mom)
- homocystinuria
136
14 yo girl with sickle cell disease presents with red swollen painful hand. No vitals mentioned.
What are three things you do for management.
After leaving the hospital, what is one medication that can be taken for prevention of these episodes
1. dactylitis mgmt:
- IV hydration
- pain control (start with acetaminophen and NSAIDs, often need opioids)
- distraction (heat pads, play therapy, massage)
- consider ix for osteomyelitis
2. hydroxyurea
137
Child described with cleft palate, TOF, low calcium.
CBC shows Hb 110, plt 100.
Baby blood type AB + (No mention of maternal plt results)
He is going for cardiac surgery. - if he has blood loss what do you do?
1. Give him irradiated blood
2. Pla1 platelets
3. Maternal washed platelets
4. O- blood
1. Give him irradiated blood
Due to immunedeficiency with diGeorge, will need leukocyte depleted, CMV negative and irradiated blood products.
138
Poor prognosis in ALL is suggested by:
a) female
b) age <1 year
c) CALLA positive
d) mediastinal mass
e) splenomegaly
b) age <1 year
CALLA positive indicative of positive response to treatment
Poor prognosis: age <1 or >10, T cell leukaemia, initial WBC >50,000, slow response to initial treatment
(female is a favourable prognosis)
139
You are referred the following 16 month old child with a suspicion of non-accidental trauma (bilateral periorbital ecchymoses).
On examination you find that the child has nystagmus and a palpable abdominal mass. What is the most likely diagnosis?
Neuroblastoma
Sounds like she has opsoclonus-myoclonus
- Primary tumor locations: adrenals (50%), retroperitoneal sympathetic ganglia (30%), cervical thoracic ganglio (30%)
140
Risk of HIV from transfusion of one unit pRBC?
1 in 10 million
141
Which is true of infantile hemangioma?
a. start to involute after first decade
b. are not present at birth
b. are not present at birth
| Except the congenital ones
Infantile - 60% gone by 5 years, 95% by 9 years
142
Photo of child who presents with bruising around the eyes and blue nodules, what other things will you find on examination (list 5)
(Neuroblastoma)
Hypertension
Failure to thrive
Abdominal mass (⅔ of tumors arise in abdomen)
Lymphadenopathy
Opsoclonus myoclonus (paraneoplastic syndrome)
Proptosis
Periumbilical bruising (retroperitoneal)
Scrotal swelling/lower extremity edema (from compression of venous/lymphatic systems)
Horner syndrome (ptosis, miosis, and anhidrosis) - thoracic tumor
Signs of spinal cord compression- paravertebral tumors
143
What are two life-threatening acute presentations of a teenage boy with an anterior mediastinal mass?
State the acute presentation and why it is life-threatening.
SVC Syndrome: respiratory distress
Tracheal Compression - airway compromise
Cardiac compression - tamponade, hypotension
144
List 3 side effects of Vincristine
```
Hair loss
Nausea/Vomiting
Constipation
Local cellulitis
*** Peripheral neuropathy
*** SAIDH
*** Seizures
```
145
ITP boy.
Management when platelets 25 (normal Hgb and WBC) and not bleeding?
Comes back 1 week after, platelets are 8 and Hgb 80, nosebleeds how do you managed now? (
1. If no RF: watchful waiting
| 2. Consider IVIG + Steroids, consult heme for potential BM Bx given bicytopenia.
146
Teenage boy with stage III Hodgkin’s. Going to have chemo and radiation.
1. ) List 2 factors that may affect his fertility
2. ) Give 2 options to deal with this possible infertility
1. High cumulative dose of alkylating agents, high radiation doses, health behaviour like tobacco or cigarette smoking
2. sperm banking (for boys who have gone through puberty)
- shield testicles during radiation
147
You are seeing a 14 year old who is new to your practice. She is post-lymphoma treatment with a regimen that included adriamycin, bleomycin, cyclophosphamide, vincristine and prednisone. She also received involved field radiation. She is in remission now and doing well.
A) What is a long-term side effect of bleomycin?
B) List 3 other long-term side effects of the chemo she received.
C)List one long-term effect from radiation.
A) Pulmonary fibrosis and impaired lung function
B) Adriamycin: cardiomyopathy
Cyclophosphamide: infertility (in males), bladder cancer, secondary neoplasms
Vincristine: persistent peripheral neurologic compl
Prednisone: growth suppression, cataracts, reduced bone mineral density
C) Impaired growth (from cranial or vertebral irradiation)
Endocrine dysfunction (from midbrain irradiation)
Pulmonary or cardiac insufficiency (from chest irradiation)
Strictures and adhesions (from abdo irradiation)
Infertility (from pelvic irradiation)
Second malignancy → ex. Breast cancer from chest irradiation, brain tumors from CNS irradiation
148
Neutropenic child with central line site red.
| Which antibiotics?
piperacillin-tazobactam and vancomycin
149
A patient with comes in with a mass palpable between the left lower rib and pelvic bone. Hb is 40 and he is in respiratory distress. What type of crisis is this?
Splenic sequestration
150
Give 4 mechanisms of anemia in a child with chronic renal disease.
Decreased EPO production
Iron deficiency due to chronic blood loss (venous samples, surgery, dialysis)
Higher hepcidin levels (filtered by glomerulus and excreted by kidney. Hepcidin is a regulatory protein the controls intestinal iron absorption and tissue distribution)
Inflammation -> decreased RBC lifespan
Hyperparathyroidism
Deficiencies of folate, B12, carnitine
Impared iron metabolism (iron diverted into reticuloendothelial system = functional iron deficiency)
151
An 8 month old boy presents with petechiae. On the examining table he has a bloody stool. He also has a history of eczema, pneumonia and an otitis media. His lab work shows an elevated IgE, and IgA. He has a platelet count of 80 and a coombs positive anemia. What is his diagnosis?
1. Hemophilia
2. HSP
3. Wiskott Aldrich
3. Wiskott Aldrich
152
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
b. acute cholecystitis
153
```
2 year old presents with progressive pallor over the last few weeks. You discover his Hb is 48, with a reticulocyte count of 1%. He drinks 1 L of homo milk a day. Because he has no risk factors on history or physical, you do some bloodwork which shows the following:
• MCV 80 normocytic
• HB electrophoresis: AS
• Blood smear: normal
What is the most likely diagnosis?
a. sickle cell disease
b. congenital red cell aplasia
c. transient erythroblastopenia of childhood
```
c. transient erythroblastopenia of childhood
* • HB electrophoresis: AS sickle cell trait -> typically asymptomatic
TEC
- Previously healthy kids 6mos-3years, often after a virus
- temporary suppression of erythropoiesis with reticulocytopenia and moderate to severe NORMOcytic anemia
(Neutropenia in 20% of cases)
154
Long term complications of curative therapy for cancer. List 4.
- cardiomyopathy
- hypothyroidism
- infertility
- pulmonary fibrosis
155
Kid with spherocytosis, URTI symptoms, now has a hbg of 40 (baseline is normal), low retics relative to low hbg, high bilirubin.
What is the cause?
Aplastic Crisis secondary to parvovirus or hypo plastic crisis from another infection
156
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:
1. BMA
2. Verify compliance
3. Hb electrophoresis
4. Jejunal biopsy
2. Verify compliance
157
Young kid with behaviour change, microcytic, hypochromic anemia with basophilic stippling. Physician has already done iron studies, which were normal. What would you test?
Lead
Toxic levels of other heavy metals like zing can produce stippling, but it is much more rare
Megaloblastic anemia due to vitB12/folate def or chonic alcohol use can also have basiphilic stippling
158
Better prognosis in neuroblastoma is associated with:
a) female
b) age less than 1 year
c) high excretion of VMA
d) normal blood pressure
e) unilateral
b) age less than 1 year
159
A 4-year-old child has a chest x-ray done for a different reason, but it shows an asymptomatic solid circular lesion in the anterior mediastinum:
a) ganglioneuroma
b) neuroblastoma
c) lymphoma
d) teratoma
e) metastasis from a Wilms' tumor
Lymphoma or Teratoma
Teratoma should have classic findings of fat/calcification
Lymphoma should present with B symptoms
```
DDX for anterior mediastinal mass:
Teratoma (fat/calcification)
Thymoma
Terrible lymphoma
Thyroid mass
```
160
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. von Willebrands
Factor V Leiden mutation predisposes to clots, not bleeding
161
The most common bug in febrile neutropenia:
pseudomonas
PCP
gram positive organisms
candida
Gram Positive Organisms
- staphylococcus aureus most common, then gram negative bacteria
162
A young child has an adrenal mass and hepatomegaly on ultrasound. What is the one test most likely to give you the diagnosis?
a) urine HVA, VMA
b) MIBG scan
c) CT head, neck, chest, and abdomen
a) urine HVA, VMA
(homovanillic and vanillylmandelic)
Evaluation for metastatic disease should include CT or MRI of chest and abdomen
MIBG should be used to better define extent of disease
163
2 life threatening presentations of anterior mediastinal mass?
- SVC syndrome (facial or upper extremity swelling from vascular compression)
- cardiac tamponade
- respiratory arrest from airway compression
164
List 3 side effects of L-aparaginase
```
Fever + Chills
Nausea + Vomiting
*** Allergic reaction
*** Pancreatitis
***Liver failure
Encephalopathy
Hyperglycaemia
Platelet dysfunction
Coagulopathy
```
165
In which of the following is platelet transfusion indicated?
a. aplastic anemia
b. acute ITP
c. chronic ITP
d. HUS
e. ITP secondary to quinidine toxicity
a. aplastic anemia
Avoid in consumptive processes. *note: give in acute ITP if intracranial bleed
166
Child with Wilm’s tumor. Which is associated?
a. Down syndrome
b. Prader-Willi syndrome
c. Beckwith-Weideman
d. Angelman’s syndrome
c. Beckwith-Weideman
167
A girl presents with Hb=80 and MCV=60. Ferritin and iron are normal. Next test:
a. Hb electrophoresis
b. Barium swallow
c. Bone marrow
a. Hb electrophoresis
```
Thalassemia
Anemia of Chronic Disease
Iron-deficiency
Lead-poisoning
Sideroblastic anemia
```
168
Child with bloody diarrhea, anuria, petechiae on lower legs. What is diagnosis. What is the pathophysiology of the anemia?
1. Hemolytic Uremic Syndrome
| 2. Microangiopathic Hemolytic anemia (RBCs hemolyzed as they travel through microvasculature)
169
List 3 long term effects of cyclophosphamide
- bladder cancer
- delayed puberty
- infertility
- premature menopause
- AML
- pulmonary fibrosis
170
Kid with thalessemia trait. Most likely CBC findings.
Microcytic anemia with increased RBCs
171
Frequency of Abdominal Ultrasound in Beckwith-Wiedemann Syndrome
Abdo US q3mos until 4 years (for hepatoblasoma), then RUS q3mos 4-8 years (Wilms)
AFP q3mos until 4 years.
172
Term baby, Asian descent.
Mom AB +ve, baby B +ve.
Hgb 105.
What is the likely cause?
A) Thalassemia
B) ABO incompatibility
C) G6PD
c) G6PD
Mom doesn't A or B antibodies, so cannot be ABO
173
14 year old female presents with pain above the right knee which is 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:
osteomyelitis
osteosarcoma
osteoid osteoma
subperiosteal hematoma
Osteosarcoma
174
Neutropenic child with red central line site.
| What antibiotics?
Pip-tazo and Vanco
175
Tumor lysis syndrome. On alloprirunol urines ph is 7.5. Hyperhydrated. What to do next.
a) potassium in IV fluids
b) add rasburicase
b) add rasburicase
176
A 7 year old girl comes to see you with decreased energy and pallor for the past week. She had a viral illness 1-2 weeks ago. You notice that her sclera seem a bit yellow. Her CBC shows a Hgb 70, normal WBC, normal platelets, and a retic count of 24%. What type of anemia is this [1 point]? What test would you do to confirm [1 point]? What treatment could you offer [1 point]?
1. autoimmune hemolytic anemia
2. DAT
3. Hb <100 and retics >10% - consider prednisone and IVIG
