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
