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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
Q

subacute side effects of radiation

A
  • somnolence syndrome
  • alopecia
  • radiation recall
  • photosensitivity
  • skin rash/hyperpigmentation
  • radiation necrosis
26
Q

4-5 late effects of radiation

A

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
Q

pilocytic astrocytoma WHO grade 1:

  • name molecular finding
  • other conditions that also have this molecular finding
A

BRAF fusion or V600E

  • Melanoma
  • Lung cancer
  • LCH
28
Q

Methotrexate-induced encephalopathy - how to prevent this?

A
  • use of leucovorin
  • hyperhydration
  • delay next dose of IT methotrexate
  • consider dextromethorphan
  • aminophylline
29
Q

What are the Health Canada practitioners expectations for the SAP application

A

failure of conventional medications

  • proof of efficacy and safety
  • accountable for use of drug to manufacturer and SAP
30
Q

2 distinct mechanisms by which hyperleukocytosis occurs?

A

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
Q

Good risk cytogenetics for AML

A
  • inv 16
  • t(8;21)
  • t(15;17)
  • MLL (1;11) only
32
Q

List 3 indications that you would give reduce intensity conditioning instead of myeloablative?

A
  • 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
Q

3 likely causes for hyperbili D10 post transplant

A
  • VOD
  • liver injury from CSA/Flu
  • CMV
  • GvHD
34
Q

List 4 risk factors for chronic GVHD

A

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
Q

Poor risk cytogenetics for AML

A
  • monosomy 7
  • del 5q
  • flt-3 ITD
  • MLL (10;11), (6;11)
36
Q

Medulloblastoma.: what are 3 non-genetic prognostic factors

A
  • Age
  • Extent of resection
  • Histology (large cell/anaplastic)
  • Metastatic disease
37
Q

Adolescents have worse outcomes than younger children with the same tumors. List 3 possible explanations other than biology

A
  • 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
Q

3 reasons why LMWH better than standard heparin

A
  • 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
Q

3 prognostic factors in relapsed Wilms

A

time to relapse < 12 m. is bad

  • histology – anaplasia is bad
  • previous exposure to anthracycline
  • presence of metastatic disease
  • complete resection
40
Q

4 late effects of Wilms Tumor treatment and their aetiology

A

cardiac failure from anthracycline exposure

  • hypertension from renal dysfunction
  • scoliosis from flank radiation
  • secondary malignancy from radiation or chemo
  • infertility for females.
41
Q

Name 2 of the three AIDS-defining malignancies and their associated oncogenic viruses

A
  • Kaposi sarcoma: HHV8
  • aggressive b-cell NHL: EBV
  • cervical cancer and HPV
  • leiomyosarcoma HIV
42
Q

3 strategies to conserve platelets.

A

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
Q

CML response times

A
  • normal counts by 3 m
  • normal cytogenetic response by 6-12 m
  • normal molecular response by 12-18 m
44
Q

2 cytogenetic alterations found in leukemia of ambiguous lineage

A
  • MLL

- bcr-abl

45
Q

What is the definition of a massive transfusion

A

10 U in 24 hours, OR >3 in 1 hour

46
Q

4 clinical or laboratory findings you would expect in a massive transfusion?

A
  • hyperkalemia
  • hypocalcemia
  • low fibrinogen
  • prolonged INR/PTT
  • hypothermia
  • volume overload
  • transfusion reaction
  • alloimmunization
47
Q

4 components of cryoprecipitate?

A
  • FVIII, vWF, FXIII, fibrinogen
48
Q

Cancers seen in BWS

A
Wilms
Hepatoblastoma
Neuroblastoma
rhabdomyosarcoma
Adrenocorical carcinoma
49
Q

Li-Fraumeni tumors

A
ALL
Any sarcoma except Ewings
Breast
HGG and medullo
ACC
50
Q

Gorlin syndrome tumors

A

Medullo
Basal cell carcinoma
rhabdo/myogenic tumors

51
Q

NF-1 tumors

A

OPG
neurofibromas
Sarcoma: rhabdo, MPNST, GIST, glomus tumor

52
Q

DICER-1 tumors

A
pleuropulmonary blastoma
Sertoli-Leydig tumors
multi-nodular goiter
Cystic nephroma/Wilms
embryonal rhabdo of cervix
53
Q

Adverse risk factors for prognosis in neuroblastoms

A
Low HVA-VMA ratio
Myc-N amplification
unfavorable INPC (undifferentiated histology)
Segmental chromosomal aberrations
Diploid dna content
54
Q

Risk factors for developing PTLD

A
  • type of transplant
  • severity of immunosuppression
  • EBV positive transplant in EBV neg donor
  • young age
  • malignancy pre-transplant
55
Q

Primary CNS small round blue cell tumors

A
Medulloblastoma
ATRT
Medulloepithelioma
CNS neuroblastoma
CNS ganlioneuroblastoma
ETMR

pineoblastoma
retinoblastoma
CNS lymphoma

56
Q

INI negative tumors

A

CNS ATRT
rhabdoid tumor of kidney
SCU hepatoblastoma

57
Q

Which cell types are targeted by Alemtuzumab

A

anti CD 52

B cell
T cell
NK cell
monocyte
dendritic cell
58
Q

2 most common HLH mutations in Caucasian

A

Perforin

MUNC12D

59
Q

Head & Neck manifestations of myeloablative vs RIC regimen

A
  • Severe mucositis
  • Seizure from high dose Busulfan
  • Alopecia
  • Severe emesis
  • Skin rash/darkening
  • Dysphagia
  • Dysgeusia: change in taste
  • Dental caries
  • xerostomia
60
Q

four modifications made to standard therapy when treating patients with Down syndrome ALL

A
  • 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
Q
EOL symptom management:
Pain
anxiety
nausea
dyspnea
A

pain: breathing, meditation, massage, opiods, non-opiods
anxiety: benzo, SSRI
nausea: ondans, nabilone
dyspnea: benzo, opioid, fan

62
Q

Chronic organ effects of iron overload

A
  • 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
Q

Steps to prevent TRALI

A
  • leukoreduction of blood products
  • HLA-matched products
  • Plasma reduce product
  • Solvent detergent plasma instead of ffp
  • Avoid multi-parous donor
  • Younger donors
64
Q

four factors that should be considered in determining whether someone should be credited as an author on a journal article?

A
  • 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
Q

MIBG s/e

A
Hypothyroidism
Myelosuppression
N/V
Parotid swelling
LFTs
infections
66
Q

INI negative tumors

A
ATRT
rhabdoid tumor of the kidney
SCU hepatoblastoma
extra-renal rhabdoid tumor
epithelioid sarcoma