1
Q
JMML criteria
A
Criteria: monocytosis > 1.5, splenomegaly, blast count < 20%, no bcr-abl
Genetics: NF-1, PTPN-11, N-ras, K-ras, cbl
Other: elevated Hb F, increased myeloid precursors, hypersensitivity to GM-CSF
Associated syndrome: Noonan’s and PTPN11
2
Q
tumor lysis syndrome – 2 drugs to treat and their mechanisms of action
A
Allopurinol – xanthine oxidase inhibitor. Prevents further production of urate
Rasburicase – urate oxidase. Coverts urate to allantoin which can be voided
3
Q
Translocations for: Alveolar rhabdo: Synovial sarcoma: DSRBCT: ALCL Ewings:
A
Alveolar rhabdo: t(2;13) Synovial sarcoma: t(x;18) DSRBCT: t(11;22) ALCL: t(2;5) Ewings: t(11;22)
4
Q
3 ways to differentiate hypoplastic MDS from idiopathic aplastic anemia
A
MDS has:
- patchy clusters of erythroid precursors
- Dysplastic megakaryocyte (micromegs)
- Marked decrease in granulocytes with left shift
5
Q
4 mechanisms of action of hydroxyurea
A
- Increased production of Hb F
- Increased NO production
- Lowers WBC count
- Inhibition of ribonucleotide reductase
6
Q
4 inherited conditions that predispose to OS
A
- P53
- Rb
- Bloom
- Rothmund-Thompson
- Werner
7
Q
premedications for Ch.14.18
A
- Tylenol
- morphine
- Benadryl
- albumin
- ranitidine
- IV fluids
- consider hydroxyzine or certirizine
- consider gabapentin
8
Q
List 3 patient-related factors associated with inhibitors in hemophilia A
A
- severity of hemophilia
- genetic mutation
- family history
- race
9
Q
3 treatment-related factors associated with inhibitors in hemophilia A
A
- intensity of therapy
- recombinant factor is higher risk than plasma derived
- number of exposure days
- cumulative exposure
10
Q
4 specific influential factors for successful immune tolerance in Hemophilia A?
A
- severity of inhibitor at the start of ITI
- peak inhibitor titre
- frequency of ITI administration
- use of high-dose rather than low-dose regimen (results similar but less bleeding)
- younger patients
- compliance
11
Q
mechanism of retinoic acid
A
Induces differentiation and growth arrest of NB cells
12
Q
Two most concerning bacteria for F&N
A
MRSA (?strep viridans)
Pseudomonas
13
Q
Identify 2 subtle CT signs suggestive of invasive aspergillosis.
A
- halo sign
- air-crescent sign
14
Q
3 ethical principles of TCSP2
A
Respect for persons
Concern for welfare
Justice
15
Q
5 causes of severe (life-threatening) transfusion-related events that are reportable
A
- TRALI
- Acute hemolytic reaction
- anaphylaxis
- bacterial contamination
- wrong labeling
- TA-GVHD
note that TACO is not one of them
16
Q
DIPG: 3 management strategies
A
- 54Gy radiation to prolong survival
- Involve palliative care service
- Dex to prevent cerebral edema
17
Q
3 ways to obtain a sperm sample for cryopreservation
A
- Manual ejaculation
- Trans-rectal Electro-ejaculation
- Percutaneous epididymal sperm aspiration
- Testicular biopsy – investigation.
18
Q
Isolated pulmonary nodule in. What to do
- Ewing’s
- Osteo
- Hodgkin’s
A
- Ewing’s: rads
- Osteo: resection
- Hodgkin’s: systemic chemo with auto transplant
19
Q
What features at ALL relapse make it high risk?
A
- Time from relapse < 18 months for CNS/testicular, <36 m for BM)
- Immunophenotype (T worse than B)
- Extent of disease (CNS>combined>isolated marrow)
- Age of patient
- WBC count
- MRD at relapse
Cytogenetics
Down syndrome
20
Q
Acute radiation side effects
A
- Skin rash
- Mucositis
- Cytopenias
- Alopecia
- Xerostomia
- Nausea and vomiting
- paresthesias
- cerebral edema
21
Q
. Ependymoma: name 3 prognostic factors
A
- extent of resection
- age (and ability to radiate
- molecular genotype (REL-A bad)
- histology (anaplasia bad)
- metastatic disease
22
Q
3 translocations in EWS
A
(11;22)
(21;22)
(7;22)
23
Q
Posterior-fossa syndrome
- natural history
- long-term sequelae
- prognostic factor
A
- natural history: 50% recovery
- long-term sequelae: neuro-cog delay
- prognostic factor: initial severity
24
Q
are 3 leading causes of death at 15-20 years post Hodgkin’s lymphoma
A
- breast cancer
- thyroid
- colorectal
- cardiomyopathy
25
subacute side effects of radiation
- somnolence syndrome
- alopecia
- radiation recall
- photosensitivity
- skin rash/hyperpigmentation
- radiation necrosis
26
4-5 late effects of radiation
cognitive dysfunction
- poor school performance
- endocrinopathy
- poor bone growth
- challenges with independent living
- poor social adjustment
- vasculopathy – Moya Moya disease
- infertility depending on field
27
pilocytic astrocytoma WHO grade 1:
- name molecular finding
- other conditions that also have this molecular finding
BRAF fusion or V600E
- Melanoma
- Lung cancer
- LCH
28
Methotrexate-induced encephalopathy - how to prevent this?
- use of leucovorin
- hyperhydration
- delay next dose of IT methotrexate
- consider dextromethorphan
- aminophylline
29
What are the Health Canada practitioners expectations for the SAP application
failure of conventional medications
- proof of efficacy and safety
- accountable for use of drug to manufacturer and SAP
30
2 distinct mechanisms by which hyperleukocytosis occurs?
high blast count leads to sludging in the microcirculation and hence poor oxygenation of local tissue
- high metabolic rate of blasts also leads to tissue hypoxia
- stickier cells leads to obstruction of microcirculation
31
Good risk cytogenetics for AML
- inv 16
- t(8;21)
- t(15;17)
- MLL (1;11) only
32
List 3 indications that you would give reduce intensity conditioning instead of myeloablative?
- poor ECOG score
- concurrent infections
- underlying genetic condition that prevents use of myeloablative conditioning – ex. FA
- previous transplant
- Down syndrome
- marrow aplasia pre-transplant
33
3 likely causes for hyperbili D10 post transplant
- VOD
- liver injury from CSA/Flu
- CMV
- GvHD
34
List 4 risk factors for chronic GVHD
acute GvHD
- HLA-mismatch
- stem cell source (ex. Pbsc > cord_
- length of immunosuppression?
- organ injury/infection prior to transplant
- older recipient
- CMV
- female to male
35
Poor risk cytogenetics for AML
- monosomy 7
- del 5q
- flt-3 ITD
- MLL (10;11), (6;11)
36
Medulloblastoma.: what are 3 non-genetic prognostic factors
- Age
- Extent of resection
- Histology (large cell/anaplastic)
- Metastatic disease
37
Adolescents have worse outcomes than younger children with the same tumors. List 3 possible explanations other than biology
- Worse compliance
- Increased toxicity with therapy
- Delay in seeking medical attention
- Less likely to be enrolled in clinical trial
- Treated on adult protocol or in adult center
38
3 reasons why LMWH better than standard heparin
- better PK
- no need for IV access
- outpatient management
- better correlation between dose and anticoagulant response – allows fixed dose without monitoring
- lower risk of HIT
- less osteoporosis
39
3 prognostic factors in relapsed Wilms
time to relapse < 12 m. is bad
- histology – anaplasia is bad
- previous exposure to anthracycline
- presence of metastatic disease
- complete resection
40
4 late effects of Wilms Tumor treatment and their aetiology
cardiac failure from anthracycline exposure
- hypertension from renal dysfunction
- scoliosis from flank radiation
- secondary malignancy from radiation or chemo
- infertility for females.
41
Name 2 of the three AIDS-defining malignancies and their associated oncogenic viruses
- Kaposi sarcoma: HHV8
- aggressive b-cell NHL: EBV
- cervical cancer and HPV
- leiomyosarcoma HIV
42
3 strategies to conserve platelets.
defer elective surgeries
- limit transfusions to 1 unit instead of 15 ml/kg
- restrict transfusions to bleeding patients rather than prophylaxis
- Split available aliquots
- Identify platelets near expiry to minimize waste
43
CML response times
- normal counts by 3 m
- normal cytogenetic response by 6-12 m
- normal molecular response by 12-18 m
44
2 cytogenetic alterations found in leukemia of ambiguous lineage
- MLL
| - bcr-abl
45
What is the definition of a massive transfusion
10 U in 24 hours, OR >3 in 1 hour
46
4 clinical or laboratory findings you would expect in a massive transfusion?
- hyperkalemia
- hypocalcemia
- low fibrinogen
- prolonged INR/PTT
- hypothermia
- volume overload
- transfusion reaction
- alloimmunization
47
4 components of cryoprecipitate?
- FVIII, vWF, FXIII, fibrinogen
48
Cancers seen in BWS
```
Wilms
Hepatoblastoma
Neuroblastoma
rhabdomyosarcoma
Adrenocorical carcinoma
```
49
Li-Fraumeni tumors
```
ALL
Any sarcoma except Ewings
Breast
HGG and medullo
ACC
```
50
Gorlin syndrome tumors
Medullo
Basal cell carcinoma
rhabdo/myogenic tumors
51
NF-1 tumors
OPG
neurofibromas
Sarcoma: rhabdo, MPNST, GIST, glomus tumor
52
DICER-1 tumors
```
pleuropulmonary blastoma
Sertoli-Leydig tumors
multi-nodular goiter
Cystic nephroma/Wilms
embryonal rhabdo of cervix
```
53
Adverse risk factors for prognosis in neuroblastoms
```
Low HVA-VMA ratio
Myc-N amplification
unfavorable INPC (undifferentiated histology)
Segmental chromosomal aberrations
Diploid dna content
```
54
Risk factors for developing PTLD
- type of transplant
- severity of immunosuppression
- EBV positive transplant in EBV neg donor
- young age
- malignancy pre-transplant
55
Primary CNS small round blue cell tumors
```
Medulloblastoma
ATRT
Medulloepithelioma
CNS neuroblastoma
CNS ganlioneuroblastoma
ETMR
```
pineoblastoma
retinoblastoma
CNS lymphoma
56
INI negative tumors
CNS ATRT
rhabdoid tumor of kidney
SCU hepatoblastoma
57
Which cell types are targeted by Alemtuzumab
anti CD 52
```
B cell
T cell
NK cell
monocyte
dendritic cell
```
58
2 most common HLH mutations in Caucasian
Perforin
| MUNC12D
59
Head & Neck manifestations of myeloablative vs RIC regimen
- Severe mucositis
- Seizure from high dose Busulfan
- Alopecia
- Severe emesis
- Skin rash/darkening
- Dysphagia
- Dysgeusia: change in taste
- Dental caries
- xerostomia
60
four modifications made to standard therapy when treating patients with Down syndrome ALL
- Dose of methotrexate is lower (intermediate instead of HD)
- Leucovorin rescue with IT methotrexate
- IVIG replacement
- Admission in induction until count recovery
- Prophylactic anti-fungal therapy
61
```
EOL symptom management:
Pain
anxiety
nausea
dyspnea
```
pain: breathing, meditation, massage, opiods, non-opiods
anxiety: benzo, SSRI
nausea: ondans, nabilone
dyspnea: benzo, opioid, fan
62
Chronic organ effects of iron overload
- Cardiomyopathy
- Bronzening of the skin from iron deposition
- Liver fibrosis/cirrhosis
- Hypothyroidism
- Cytopenias
- Diabetes from pancreatic failure
- Hypogonadism – decreased libido and impotence, delayed puberty
- Arthropathy
63
Steps to prevent TRALI
- leukoreduction of blood products
- HLA-matched products
- Plasma reduce product
- Solvent detergent plasma instead of ffp
- Avoid multi-parous donor
- Younger donors
64
four factors that should be considered in determining whether someone should be credited as an author on a journal article?
- substantial contributions to conception or design of work OR acquisition/analysis/interpretation of data
- Drafting/critical revision of manuscript
- Final approval of version to be published
- Agreement to be accountable for all aspects of the work
65
MIBG s/e
```
Hypothyroidism
Myelosuppression
N/V
Parotid swelling
LFTs
infections
```
66
INI negative tumors
```
ATRT
rhabdoid tumor of the kidney
SCU hepatoblastoma
extra-renal rhabdoid tumor
epithelioid sarcoma
```
SADL Oncology
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