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Heme/Onc Boards > Random Questions from Practice Tests > Flashcards

Random Questions from Practice Tests Flashcards

1
Q

Mechanism of Action for rolapitant

A

substance P/neurokinin-1 inhibitor

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2
Q

Risk factors for adenocarcinomas of the nasal cavity/sinuses

A

Wood dust and leather dust

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3
Q

Cancer thorotrast is associated with

A

cholangiocarcinoma

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4
Q

Conslidation strategy after induction for CNS lymphoma

A

auto SCT or whole brain RT

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5
Q

Drugs in CHOP

A

cyclophosphamide, doxorubicin, vincristine, prednisone

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6
Q

Effects of TKI in pregnancy

A

increases fetal malformation and spontaneous abortions

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7
Q

In what position does SVC syndrome worsen?

A

Supine

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8
Q

What is the presenting triad associated with Plummer-Vinson syndrome, and what cancer is it associated with?

A

Iron deficiency anemia, esophageal webs, dysphagia. Patients are at increased risk of developing Squamous cell carcinoma of the esophagus

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9
Q

Appropriate duration of anticoagulation in women who have DVT during pregnancy

A

6 weeks after birth

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10
Q

When should chemotherapy be added to adjuvant radiation in patients with head and neck cancer?

A

Positive margins or extracapsular extension

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11
Q

Hct goal post cardiac surgery

A

24%

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12
Q

What are the initiator caspases?

A

2, 8, 9, 10

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13
Q

First line therapy for BRAF WT Melanoma

A

Pembrolizumab or Ipi/Nivo

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14
Q

What are the mandatory criteria to diagnose POEMS syndrome

A

Polyneuropathy and Monoclonal plasma cell proliferative disorders (almost always lambda)

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15
Q

What does POEMS stand for?

A

Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, and Skin changes

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16
Q

What are the major criteria for POEMS and how many do you need for diagnosis?

A

You need one. Castelman disease, sclerotic bone lesions, and VEGF elevation

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17
Q

Appropriate treatment for locally advanced BCC that cannot be treated with surgery and radiation

A

Hedgehog pathway inhibitor (vismodegib or sonidegib)

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18
Q

How would you treat a grade 1 or 2 metastatic endometroid endometrial carcinoma not eligible for 1st line chemo?

A

Sequential megestrol and tamoxifen

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19
Q

What is the mechanism of action and indication for tositumomab?

A

Monoclonal antibody against CD20 receptor. Used to treat relapsed or treatment refractory Non-Hodgkin Lymphoma (NHL)

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20
Q

What is the mechanism of action and indication for alemtuzumab?

A

Monoclonal antibody against CD52 receptor; used in management of CLL

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21
Q

What is the mechanism of action and indication for Brentuximab vedotin?

A

Monoclonal antibody against CD30 receptor. Used in treatment of Hodgkin’s lymphoma and anaplastic large cell lymphoma

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22
Q

How do you calculate an odds ratio?

A

Odds of exposure with cases divided by odds of exposure within controls

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23
Q

What hereditary condition is associated with an increased risk fo developing desmoid tumors?

A

Gardner syndrome

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24
Q

What are patients with Cowden syndrome at risk for?

A

GI hamartomas, breast/thyroid/uterine cancer

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25
Q

How do you define unfavorable disease for early-stage Hodgkin lymphoma according to NCCN guideline?

A

Bulky mass (>10cm), large mediastinal mass (MMR >.33), ESR>50, presence of any B symptoms, or >3 nodal areas

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26
Q

What percentage of men with high-grade PIN will develop prostate cancer within 5 years?

A

50%

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27
Q

What is the optimal treatment regimen for primary mediastinal B-cell lymphoma?

A

DA-EPOCH-R x 6 cycles

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28
Q

What measure do you use to express association between an exposure and outcome in case-control studies?

A

Odds Ratio

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29
Q

What measure do you use to express association between an exposure and outcome in cohort studies?

A

Relative Risk

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30
Q

What enzyme is missing in acute intermittent porphyria?

A

Porphobilinogen deaminase

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31
Q

What is the classic translocation seen in MALT lymphoma?

A

t(11;18)

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32
Q

What is the alteration in Glanzmann’s thromabsthenia, and what is the resulting problem?

A

mutation that inactivates GP2B/3A so unable to immobilize fibrinogen and vWF

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33
Q

what is the most common genetic alteration in liposarcoma?

A

12q amplification involving MDM2 and CDK4 genes

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34
Q

what is the mechanism of action of plerixafor?

A

CXCR4 antagonist which results in the release of hematopoietic stem cells from the marrow

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35
Q

What cell surface antigens are expressed by hematopoietic stem cells?

A

CD34, CD90, CD110, CD117, CD133, CD164

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36
Q

What is the cytogenetic abnormality present in >50% of patients with hypereosinophilic syndrome?

A

del(4)(q12q12): FIP1L1-PDGFRA fusion

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37
Q

What defines early stage Hodgkin lymphoma as unfavorable?

A

Bulky disease (mediastinal mass ratio >0.33 or any mass >10cm); ESR>50; Presence of B symptoms; >3 lymphoid regions

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38
Q

What is the most commonly reported variant RARA translocation?

A

t(11;17)(q23;q21.2): PLZF-RARA

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39
Q

What does cryoprecipitate contain?

A

Fibrinogen, Factor VIII, Factor III, vWF, Fibronectin

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40
Q

What are risk factors for local recurrence in soft tissue sarcoma?

A

Age >50, Recurrent disease at presentation, Positive margins, histologic subtypes (fibrosarcoma and malignant peripheral nerve tumors)

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41
Q

What are risk factors for distant recurrence in soft tissue sarcomas?

A

Tumor size >5cm, high tumor grade, deep location, recurrent disease, leiomyosarcoma

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42
Q

What are the 4 most prominent features in dyskeratosis congenita?

A

aplastic anemia, dystrophic nail changes, reticular skin changes, lingular leukoplakia

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43
Q

In addition to aplastic anemia, what clinical findings characterize fanconi anemia?

A

developmental delay, short stature, microcephaly

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44
Q

What is the underlying pathophysiologic dysfunction in dyskeratosis congenita?

A

telomeric dysfunction (abnormally short telomeres)

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45
Q

Which CNS tumor is characterized by 1p and 19q co-deletion?

A

Oligoastrocytomas

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46
Q

Which CNS tumor frequently has an IDH1 mutation?

A

low grade astrocytoma

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47
Q

What are the peripartum anticoagulation recommendations for patients heterozygous for Factor V Leiden mutation?

A

Low dose LWMH for 6 weeks after delivery if C-section or 1st degree relative with VTE under age 50

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48
Q

Which sarcoma histologies are at high risk for lymph node metastasis?

A

CARE: clear cell, angiosarcoma, rhabdomyosarcoma, epithelioid

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49
Q

Which platelet disorder shows normal aggregation with primary and secondary ADP and collagen, but not with ristocetin?

A

Bernard-Soulier (also has large platelets)

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50
Q

Which cyclin binds to cdk4 and 6?

A

Cyclin D- to signal that cell is ready to advance to S phase from G1

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51
Q

Which cyclin regulates progression to M phase?

A

Cyclin B (by binding to CKD1)

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52
Q

What percentage of metastatic melanoma has a BRAF mutation?

A

50%

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53
Q

What is on the US black box warning for Mitomycin C?

A

hemolytic uremic syndrome

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54
Q

What are the most common sites of distant metastases for retroperitoneal sarcoma?

A

Liver (most common) followed by lung

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55
Q

What are the clinical characteristics of Gardner syndrome?

A

Multiple colyn polyps, osteomas, desmoid tumors, fibromas, thyroid cancer

56
Q

how do you calculate negative predictive value?

A

True negatives divided by total number of people testing negative

57
Q

What is the mechanism of action of ramucirumab?

A

IgG monoclonal antibody that targets VEGFR-2

58
Q

What are the two most common resistance mutations to crizotinib?

A

L1196M and G1269A

59
Q

What are the components of the international prognostic score for Hodgkin Lymphoma?

A

Age >45; WBC >15, Lymphopenia (<600 or <8% of WBCs), Stage IV, Hgb <10.5, Albumin <4.0, Male

60
Q

What genes are mutated in juvenile hemochromatosis?

A

hemojuvelin (HJV) or hepcidin (HAMP

61
Q

What is the classic MRI appearance of CNS lymphoma?

A

“cotton wool appearance”

62
Q

What is the classic MRI appearance of paragangliomas?

A

“salt and pepper” appearance

63
Q

What percentage of patients with CNS lymphoma have ocular involvement?

A

15-20%

64
Q

What is the lymphatic drainage of the upper 2/3 of the vagina?

A

pelvic nodes including internal and external iliacs, and obturator nodes

65
Q

what is the lypmhatic drainage of the lower 1/3 of the vagina?

A

inguinofemoral nodes

66
Q

Anticoagulation recommendation for pregnancy in FVL homozygous women with no personal/family hx of DVT?

A

Intermediate dose LMWH post-partum only

67
Q

Anticoagulation recommendation for pregnancy in FVL homozygous women with personal/family hx of DVT?

A

Intermediate dose LMWH during pregnancy and post-partum

68
Q

What are the 4 most common types of soft tissue sarcoma, in order?

A

1.) Undifferentiated pleomorphic sarcoma, 2.) Liposarcoma 3.) Synovial sarcoma, 4.) Leiomyosarcoma

69
Q

How many lypmh nodes should be examined in gastrectomy?

A

15

70
Q

What is the most common genetic abnormality in clear cell sarcoma?

A

t(12;22)

71
Q

What proteins in the HPV genome act as oncoproteins?

A

E7 and E8 (through destruction of p53 and Rb)

72
Q

What is the most common subtype of APL?

A

Hypergranular variant (less common is microgranular)

73
Q

What medications can be used in ACTH-secreting adenomas?

A

Ketoconazole, metrapone, mitotane

74
Q

What medications can be used in GH-secreting adenomas?

A

somatostatin analogs (somatostatin, octreotide)

75
Q

What medications can be used for prolactinomas?

A

Dopamine agonists (bromocriptine, cabergoline)

76
Q

What are the tumor marker cutoffs for good risk nonseminoma?

A

AFP <1000, B-hCG <5,000, LDH <1.5x ULN

77
Q

What are the tumor markers for poor risk nonseminoma?

A

AFP >10,000, B-hCG >50,000, LDH >10x ULN

78
Q

What is the most common mutation in NSCLC?

A

KRAS

79
Q

Treatment for early-stage unfavorable-risk HL?

A

ABVD x 4 + 30gy IFRT

80
Q

What are the non-taxane microtubule inhibitors?

A

eribulin and ixabepilone

81
Q

In what setting is pertuzumab approved in the neoadjuvant setting?

A

Tumor >2cm, positive lymph nodes

82
Q

What agent can eliminate interference of anti-CD38 targeted therapies on antibody screens?

A

DTT- but will destroy kell antigens

83
Q

What percentage of pancreatic cancer patients present with resectable tumors at baseline?

A

20%

84
Q

What percentage of pancreatic cancer patients present with metastatic disease at baseline?

A

50%

85
Q

What is the ETV6-NTRK3 fusion gene relatively pathognomonic for in salivary gland tumors?

A

mammary analogue secretory carcinoma (MASC)

86
Q

Which molecularly targeted agent would be appropriate for a patient with metastatic melanoma and an NRAS mutation?

A

Binimetinib

87
Q

What is the flow cytometry phenotype for hairy cell leukemia?

A

Positive for: CD19, CD20, CD103, CD25, CD11c

88
Q

What is the flow cytometry phenotype for splenic marginal zone lymphoma?

A

Positive for: CD20, CD79a. Negative for: CD5, CD10, CD23, CD25.

89
Q

What is first-line treatment for splenic marginal zone lymphoma?

A

single agent rituximab

90
Q

What is the most important factor associated with improved survival in patients with oligodenroglial tumors?

A

1p and 19q codeletion

91
Q

BRAF V600E is most commonly seen in what type of low grade glioma?

A

pleomorphic xanthoastrocytoma

92
Q

Treatment for 5q deleted MDS

A

Lenalidomide

93
Q

In multiple myeloma, is t(4:14) good or poor risk?

A

poor

94
Q

In multiple myeloma, is t(14:16) good or poor risk?

A

poor

95
Q

In multiple myeloma, is t(14:20) good or poor risk?

A

poor

96
Q

In multiple myeloma, are trisomies of odd number chromosomes good or poor risk?

A

good

97
Q

What are poor risk cytogenetics in multiple myeloma?

A

17p13, t(4;14), t(14;16), t(14;20)

98
Q

What cells does anaplastic thyroid carcinoma arise from?

A

follicular cells

99
Q

What condition has normal PT, PTT, TT, with positive urea clot test?

A

FXIII deficiency

100
Q

Is translocation 12;21 favorable or poor prognosis in ALL?

A

Favorable (TEL/AML1)

101
Q

Is translocation 16;16 favorable or poor prognosis in AML?

A

Favorable- can do high dose Ara-C for chemotherapy

102
Q

What are flower cells pathognomonic for?

A

adult t-cell lymphomas

103
Q

What is the flow cytometry phenotype of adult t-cell leukemia/lymphoma?

A

CD2-5, CD25. Important: lacks CD7

104
Q

Which subtype of vWD mimics hemophilia A?

A

vWD type 2N

105
Q

What malignancy is t(8;14)(q24.1;q11.2); MYC-TRA associated with?

A

T-lymphoblastic leukemia (T-ALL); carries a poor prognosis

106
Q

What is an antidote to 5-FU?

A

Uridine triacetate

107
Q

Is the absence of MGMT promotor methylation a favorable or poor prognostic indicator in primary glioblastoma?

A

Poor prognostic factor

108
Q

What is the platelet aggretometry results for Glanzmann thrombasthenia?

A

Normal aggregation with risotcetin but lack of aggregation with remaining platelet agnosits

109
Q

What is the preferred screening test for porphyria cutanea tarda?

A

Urine or plasma porphyrins

110
Q

Is wild type NPM1 and FLT3-ITD high poor risk or favorable risk in AML?

A

Adverse risk

111
Q

What AML risk category is mutated NPM1 with FLT3-ITD High

A

intermediate

112
Q

What AML risk category is mutated RUNX1?

A

Adverse

113
Q

What AML risk category is mutated ASXL1?

A

Adverse

114
Q

What AML risk category is t(6;9) DEK-NUP214?

A

Adverse

115
Q

What AML risk category is KMT2A rearranged?

A

Adverse

116
Q

What AML risk category is inv(3)?

A

Adverse

117
Q

What AML risk category is t(3;3) GATA2,MECOM(EVI1)

A

Adverse

118
Q

What AML risk category is t(8;21) RUNX1-RUNX1T1?

A

Favorable

119
Q

What AML risk category is inv(16)?

A

Favorable

120
Q

What AML risk category is biallelic mutated CEBPA?

A

Favorable

121
Q

What is enasidenib?

A

IDH2 inhibitor

122
Q

What is ivosidenib?

A

IDH1 inhibitor

123
Q

What is the pathognmonic mutation in Waldesntroms?

A

MYD88

124
Q

What is first-line therapy for metastatic cholangiocarinoma?

A

Gemcitabine/Cisplatin

125
Q

What is second-line therapy for metastatic cholangiocarcinoma?

A

FOLFOX

126
Q

Which gene is mutated in congenital gray platelet sydnrome?

A

NBEAL2

127
Q

What is the treatment for post transfusion purpura?

A

IVIG 2g/kg of body weight over 2-5 days

128
Q

What maintenance regimen is preferred in adverse risk multiple myeloma?

A

Bortezomib

129
Q

What is the mechanism of action of plerixafor?

A

CXCR4 antagonist

130
Q

Which malignancy commonly produces calcitriol?

A

Lymphoma

131
Q

What gene is mutated in Cowden syndrome?

A

PTEN (hamartomatous polyps in GI tract)

132
Q

What constitutes grade 1 diarrhea?

A

<4 bowel movements daily more than his baseline

133
Q

What is the active metabolite of irinotecan?

A

SN-38

134
Q

What does increased expression of p170 glycoprotein on cancer cells result in?

A

Increased drug efflux of many chemotherapeutic agents including irinotecan

135
Q

Which chemokine is expressed in nearly all cases of angioimmunoblastic T cell cases?

A

CXCL13 (cytoplasmic)

136
Q

What regimen should be considered in patients with CNS involvement of multiple myeloma?

A

Pomalidomide + carfilzomib + dexamethasone

137
Q

What defect is present in Hermansky-Pudlak syndomre?

A

Absence of dense granules, most common mutation in HPS1

Heme/Onc Boards (4 decks)

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