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Flashcards in Repeat exam questions Deck (66):
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

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