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1

JMML criteria

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

tumor lysis syndrome – 2 drugs to treat and their mechanisms of action

Allopurinol – xanthine oxidase inhibitor. Prevents further production of urate

Rasburicase – urate oxidase. Coverts urate to allantoin which can be voided

3

Translocations for:
Alveolar rhabdo:
Synovial sarcoma:
DSRBCT:
ALCL
Ewings:

Alveolar rhabdo: t(2;13)
Synovial sarcoma: t(x;18)
DSRBCT: t(11;22)
ALCL: t(2;5)
Ewings: t(11;22)

4

3 ways to differentiate hypoplastic MDS from idiopathic aplastic anemia

MDS has:

- patchy clusters of erythroid precursors
- Dysplastic megakaryocyte (micromegs)
- Marked decrease in granulocytes with left shift

5

4 mechanisms of action of hydroxyurea

- Increased production of Hb F
- Increased NO production
- Lowers WBC count
- Inhibition of ribonucleotide reductase

6

4 inherited conditions that predispose to OS

- P53
- Rb
- Bloom
- Rothmund-Thompson
- Werner

7

premedications for Ch.14.18

- Tylenol
- morphine
- Benadryl
- albumin
- ranitidine
- IV fluids
- consider hydroxyzine or certirizine
- consider gabapentin

8

List 3 patient-related factors associated with inhibitors in hemophilia A

- severity of hemophilia
- genetic mutation
- family history
- race

9

3 treatment-related factors associated with inhibitors in hemophilia A

- intensity of therapy
- recombinant factor is higher risk than plasma derived
- number of exposure days
- cumulative exposure

10

4 specific influential factors for successful immune tolerance in Hemophilia 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

mechanism of retinoic acid

Induces differentiation and growth arrest of NB cells

12

Two most concerning bacteria for F&N

MRSA (?strep viridans)
Pseudomonas

13

Identify 2 subtle CT signs suggestive of invasive aspergillosis.

- halo sign
- air-crescent sign

14

3 ethical principles of TCSP2

Respect for persons
Concern for welfare
Justice

15

5 causes of severe (life-threatening) transfusion-related events that are reportable

- TRALI
- Acute hemolytic reaction
- anaphylaxis
- bacterial contamination
- wrong labeling
- TA-GVHD

note that TACO is not one of them

16

DIPG: 3 management strategies

- 54Gy radiation to prolong survival
- Involve palliative care service
- Dex to prevent cerebral edema

17

3 ways to obtain a sperm sample for cryopreservation

- Manual ejaculation
- Trans-rectal Electro-ejaculation
- Percutaneous epididymal sperm aspiration
- Testicular biopsy – investigation.

18

Isolated pulmonary nodule in. What to do
- Ewing's
- Osteo
- Hodgkin's

- Ewing's: rads
- Osteo: resection
- Hodgkin's: systemic chemo with auto transplant

19

What features at ALL relapse make it high risk?

- 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

Acute radiation side effects

- Skin rash
- Mucositis
- Cytopenias
- Alopecia
- Xerostomia
- Nausea and vomiting
- paresthesias
- cerebral edema

21

. Ependymoma: name 3 prognostic factors

- extent of resection
- age (and ability to radiate
- molecular genotype (REL-A bad)
- histology (anaplasia bad)
- metastatic disease

22

3 translocations in EWS

(11;22)
(21;22)
(7;22)

23

Posterior-fossa syndrome
- natural history
- long-term sequelae
- prognostic factor

- natural history: 50% recovery
- long-term sequelae: neuro-cog delay
- prognostic factor: initial severity

24

are 3 leading causes of death at 15-20 years post Hodgkin's lymphoma

- 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