Flashcards in Repeat exam questions Deck (66)
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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
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
