Study Deck 1 Flashcards

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1
Q

Glucarpidase use indication

A

High dose MTX with kidney dysfunction

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2
Q

CXCR4 mutation

A

Waldenstroms Macroglobulinemia

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3
Q

medication used in adrenocortical tumors (adjuvant with chemo)

A

Mitotane

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4
Q

Chemo assocaited with steatohepatitis/risk before hepatic resection in colon ca

A

Irinotecan

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5
Q

Time between bevacizumab and hepatic resection

A

at least 28 days, ideally 6-8 weeks given long half life (20 days)

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6
Q

Monosomy 7 management after induction AML

A

Transplant for consolidation

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7
Q

Criteria for high risk ET

A

Age >60, hx of thrombosis or major bleeding, platelet count > 1.5 million

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8
Q

CD19+, CD22+, CD11c, CD103, CD123

A

Hairy Cell Leukemia

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9
Q

Difference between classic vs variant Hairy Cell

A

CD200+ in classic, BRAF V600E mutation uniformly positive in classic

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10
Q

Treatment of recurrent ovarian cancer-platinum resistant

A

Bevacizumab + Liposomal doxorubicin (or paclitaxel, or topotecan)

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11
Q

Treatment after recurrent ovarian cancer after CR or PR with platinum

A

Olaparib, Rucaparib, Niraparib (regardless of BRCA mutation status)

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12
Q

Inotuzumab toxicity

A

11% pts with veno-occlusive disease

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13
Q

Ponatinib toxicity

A

Arterial thrombotic disease

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14
Q

Nodular lymphocyte Hodgkin Lymphoma lacks this expression seen in classic HL

A

CD30

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15
Q

Most common testicular malignancy in men over age 60

A

primary testicular lymphoma

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16
Q

Tx of primary cutaneous follicular lymphoma (presents as non tender nodules, no ulceration)

A

respond well to radiation- EBRT for localized disease

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17
Q

Post ASCT maintenance for del17p MM

A

Bortezomib (improved PFS and OS)

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18
Q

Salvage treatment of Hpylori+ Gastric MALT lymphoma

A

Ibrutinib

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19
Q

When can bone marrow eval be omitted in MGUS/Myeloma workup?

A

IgG M spike and normal FLC ratio

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20
Q

Tazemetostat

A

EZH2 mutation, refractory follicular lymphoma

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21
Q

Oxaliplatin is an irritant or vesicant? Not given with…

A

Irritant, not given with NS or chloride containing fluids

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22
Q

Paclitxel hypersensitivity

A

Due to solvent- cremophor

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23
Q

Vandetanib

A

use in medullary thyroid cancer

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24
Q

Testing in melanoma pt not responsive to immunotherapy

A

check for ckit exon 11 or 13- option of imatinib or dasatinib

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25
Q

ISS staging for myeloma

A

Stage 1: B2 <3.5 and albumin > 3.5, Stage III: B2 >5.5

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26
Q

Cholangiocarcinoma transplant criteria

A

no lymphadenopathy, no intrahepatic or extrahepatic mets, max 3 cm lesion

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27
Q

Important CYP2D6 substrates

A

Tamoxifen and many anti-depressants

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28
Q

Cord compression Surgery vs Radiation

A

Surgery comes first, earlier walking (patchell study)

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29
Q

Adjuvant treatment for 1p/19q del oligodendroglioma

A

adjuvant PCV (procarbazine, lomustin, Vincristine) + EBRT

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30
Q

Tx for platinum refractory cervical cancer

A

Topotecan/paclitaxel/bevacizumab

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31
Q

HCC concern- cutoff for further imaging

A

10mm= 1 cm

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32
Q

Lonsurf use in advanced gastric cancer

A

TAGS trial, median OS 5.7vs 3.6 months

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33
Q

Geriatric adjuvant stage III colon cancer

A

Oxaliplatin does not necessarily have to be added to a 5-FU based regimen in elderly patients with stage III colon cancer

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34
Q

relapsed primary CNS lymphoma treatment

A

rechallenge with high dose MTX

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35
Q

when to consider adjuvant chemoRT instead of chemo for pancreatic cancer

A

subset analysis with benefit if R1 resection and + LN

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36
Q

treatment of ETV6-NTRK B-cell all

A

larotrectinib or crizotinib

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37
Q

patient with medullary carcinoma/collecting duct tumor, check what test

A

Hgb electrophoresis for sickle trait

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38
Q

Major side effect with panobinostat

A

high incidence of diarrhea

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39
Q

increased isolated PTT, normal hemophilia markers, no inhibitor

A

Factor 11 def- severe def is less than 20%. If inhibitor develops, use rFactor 7

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40
Q

Chemo response with Stage II colon cancer and MSI status

A

MSI-H Stage II- do not benefit from adjuvant chemo

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41
Q

Radiation treatment for limited stage SCLC

A

45Gy in 3 weeks (1.5 Gy BID) > 45 Gy in 5 weeks, OS benefit from CONVERT study (2017).

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42
Q

Dermatofibrosarcoma Protuberans (DFSP), metastatic treatment

A

Imatinib (ORR almost 50%), usually response if t (17,22)- fusion protein with PDGF

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43
Q

RAI indications for papillary thyroid cancer

A

Gross extrathyroidal extension, primary tumor >4 cm, postop Tg >5-10 ng/ml

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44
Q

Hereditary iron loading disorder

A

Classic Ferroportin disease, mutation in FPN- marked iron accumulation in Kupffer cells (vs HFE- hepatocytes)

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45
Q

How to modify RBCs for igA deficiency

A

Washed RBC

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46
Q

What Cr Cl to avoid Rivaroxaban

A

less than 30

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47
Q

GFR dosing for Cisplatin

A

> 60- 100% dose, 45-59- 75% of dose, <45-> consider carboplatin

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48
Q

pT2 and no nodes after cystectomy for urothelial cancer

A

No adjuvant therapy

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49
Q

Mantle Cell lymphoma markers

A

t(11,14), cyclin D1 overexpression, SOX11. Favorable if SOX 11 +

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50
Q

Imerslund Grasbeck Syndrome

A

Inherited, autosomal recessive- mutation R protein= B12 deficiency due to intestinal mucosa absorption issue, also proteinuria

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51
Q

ALK agent with best CNS activity

A

Alectinib

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52
Q

Panabinostat mechanism of action and tox

A

r/r MM progressed on bortezomib and imid, HDAC inhibitor

Black box for cardiotoxicity and diarrhea

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53
Q

Second line option with Elotuzumab aftr progression on imid and Pi

A

Elo-Pom-Dex, reminder that ELO inhibits SLAMF7= NK cell function

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54
Q

Sensorineural Hearing loss + Renal and GI malformations +Short stature+ also early onset of head and neck squamous cancer

A

Fanconi Anemia, Dx- chromosome breakage analysis

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55
Q

Bone marrow failure syndrome with elevated erythrocyte deaminase levels

A

Diamond-Blackfan= mutation in RPS19 (ribosomal)

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56
Q

Neutropenia + pancreatic insufficiency

A

Scwamann-Diamond

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57
Q

Monocytopenia + Mycobacterial Infections

A

GATA2 deficiency

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58
Q

Lab monitoring/side effect with cabozantinib

A

triglyceride eleccation

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59
Q

Immunotherapy option first line met NSCLC -benefit regardless of PDL1

A

Nivo/Ipi (CHECKMATE 227)

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60
Q

Bosutinib drug interaction that lowers plasma concentrations

A

PPI

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61
Q

response in MMR rectal cancer

A

poor response to neoadjuvant chemotherapy, so neoadjvuant chemo rads done

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62
Q

AML induction for good risk (t8,21, inv 16, NPM1+/FLT-, CEBPA x2)

A

7+3+GO

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63
Q

Not fit AML options

A

VEN+HMA, Glasdegib +LDAC

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64
Q

Splenomegaly, lymphocytosis, no lymphadenopathy, +HCV

A

Splenic marginal zone

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65
Q

Severe aplastic anemia vs very severe

A

Severe: BM cellularity <30%, 2 of 3 PB criteria- ANC 400-200, plt <20, Retic <40,000. Very severe= ANC <200

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66
Q

Bilirubin cutoff for not using irinotecan

A

2

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67
Q

management of isolated ovarian lesions <8 cm in premenopausal women

A

US to see if high risk features, can then do OCP to see if regrression

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68
Q

management of residual mass >3 cm after adjuvant treatment for seminoma

A

PET scan > if +-> resection or biopsy. If residual seminoma on that-> adjuvant chemo (VIP, TIP)

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69
Q

diagnosis of inv16 in bone marrow specimen

A

automatic classification as AML

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70
Q

Mast cell leukemia with CKIT D816V, resistance to

A

imatinib

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71
Q

RBC surface protein combination NOT susceptible to Parvo B19

A

Patients that are homozygous for pp are not susceptible to parvovirus B 19 infection.

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72
Q

what levels of ferritin and transferrin prompt hemachromatosis eval

A

Transferrin >45%, Ferritin >200

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73
Q

Platelet based bleeding disorders + Family hx of AML/MDS

A

RUNX1

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74
Q

AML with propensity for CNS dx and extramedullary hematopoiesis

A

M4

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75
Q

Follicular lymphoma transformation rate

A

3% a year

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76
Q

lymphoma with popcorn cells, CD19+, CD20+ , CD45, PAX5,, negative CD15 and NCD30

A

nodular lymphocyte predominant HL

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77
Q

RBC surface antigen associated with development of cold agglutinin disease in mycoplasma

A

Big I antigen

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78
Q

tx for adjuvant stage Ib1, B2 and IIA1 cervical cancer

A

adjuvant chemo with platinum, consideration of vaginal brachytherapy

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79
Q

commonly associated dialysis related amyloidosis

A

synovial joint space

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80
Q

treatment option for TNBC neoadjuvant not candidates for anthracycline

A

carboplatin/docetaxel

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81
Q

high risk head and neck squamous cancer

A

cisplatin 100 mg/m2 + 70 gy (7 weeks)

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82
Q

adjuvant chemotherapy for high risk uterine carcinosarcoma

A

ifosfamide/paclitaxel

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83
Q

Raloxifene vs Tamoxifen in reducing risk of bca

A

raloxifene is not as effective as tam, but less toxic (less thromboembolic events, less uterine cancer)

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84
Q

ALK mutation resistance to alectinib and brigatinib

A

G1202R

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85
Q

Timeline of ATRA differentiation syndrome

A

usually starts 7-12 days after-can be early (within 1 week), or late (up to 3 weeks later)

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86
Q

Major proteins regulated by VHL

A

HIF1a and HIF2a

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87
Q

Cowden’s syndrome

A

mutation in PTEN, predisposed to endometrial cancer, thyroid cancer, macrocephaly

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88
Q

BRCA woman and screening

A

Annual Breast MR Age 20 age 30-75- annual mammo + MRI. BSO age 35-40 (BRCA 2 onset ovarian ca 8-10 y after BRCA 1)

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89
Q

CK20 pos, Merkel Cell treatment first line-advanced disease

A

Avelumab

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90
Q

treatment for metastatic cutaneous squamous cell carcinoma

A

Cemiplimab- anti= PD1

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91
Q

Plasmacytoid urothelial carcinoma mutation

A

CDH1 mutation- aggressive variant

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92
Q

GIST mutation- best prognosis and poor prognosis

A

Best prognosis- ckit exon 11, resistance- PDGFR exon 18 D842V (resistance to imatinib, sunitib, regorafenib). C-kit exon 9 and wild type also resistance to imatinib- can try higher dose

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93
Q

Safe drugs during pregnancy

A

doxorubicin, cyclophosphamide

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94
Q

treatment of inflammatory breast cancer after clinical response

A

Sentinel LN biopsy not recommended even if complete response, usually total mastectomy + axillary dissection

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95
Q

MLH1 promoter methylation and Lynch syndrome

A

rarely associated, more likely sporadic if methylation

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96
Q

mutation associated with heriditary pancreatitis and risk of pancreatic adenocarcinoma

A

PRSS1

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97
Q

Use of dexrazoxane

A

can use if anthracycline working but concern for cumulative dosing

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98
Q

Use of pembrolizumab in late line esophageal cancer

A

third line, CPS >1

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99
Q

Key feature distinguishing fatty liver of pregnancy and HELLP

A

both can have high liver enzymes and thrombocytopenia, fatty liver tends to have hypoglycemia

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100
Q

First line treatment of metastatic cervical cancer

A

Cisplatin, Paclitaxel, Bevacizumab

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101
Q

Most common somatic mutations in uveal melanoma

A

GNAQ and GNA11

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102
Q

Mutation germline with cutaneous ad uveal melanoma

A

BAP1

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103
Q

second line endometrial cancer- testing needed

A

MMR IHC- found up to 30% of endometrial cancer

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104
Q

Adjuvant treatment of uterine confined leiomyosarcoma

A

high chance of recurrece but adjuvant chemo or radiation not associated with improved outcomes

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105
Q

Synovial sarcoma treatment

A

Sensitive to high dose ifosfamide

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106
Q

Treatment of osteogenic osteosarcoma

A

Intense chemo with MAP-> surgery-> additional MAP. NO ROLE OF RADIATION IN CURATIVE SETTING

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107
Q

Treatment of osteosarcoma older than 40

A

Cisplatin/Doxorubicin- avoiding MAP chemo (high dose methotrexate)

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108
Q

Complete staging for ovarian cancer

A

Laparotomy + omentectomy + peritoneal biopsies + LN sampling

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109
Q

what to do if some residual disease on Day 14 marrow of AML

A

80% of AML patients with day 14 marrow residual leukemia (>5%) could convert to morphological CR without re-induction on a repeated day 28 marrow.

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110
Q

worsening pain- management for already on opiods with pancreatic cancer

A

celiac plexus block

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111
Q

AML induction in those with impaired cardiac function

A

use fludarabine instead of dauno/ida- can still use combo with cytarabine for curative intent

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112
Q

Adjuvant treatment GBM

A

RT + Temozolomide for 6 weeks, then adjuvant 6 cycles TEM + TTF (TTF addition adds 4.9 months for median survival)

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113
Q

Workup of CML

A

Bone marrow still needed given need % blasts in marrow, cytogenetics- to distinguish phases of CML

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114
Q

opioid for renal dysfunction

A

fentanyl does not have active metabolites excreted by kidneys. also can use hydromorphone and methadone

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115
Q

treatment of post-radiation angiosarcomas

A

mastectomy/surgery with wide margins

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116
Q

chemotherapy effective as late line agent for dedifferentiated liposarcomas

A

eribulin

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117
Q

TKI effective in advanced soft tissue sarcomas

A

pazopanib

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118
Q

most common malignancy in testes in age >60

A

NHL

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119
Q

first line tx for multicentric castlemans

A

siltuximab + dex

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120
Q

Bone marrow biopsy criteria for M spike

A

M protein 1.5 g/dl or higher, any non-IgG MGUS, abnormal FLC ratio (>1.65)

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121
Q

localized DLBCL in gastric area tx

A

avoid surgery in general, chemo with RCHOP

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122
Q

bilateral adrenal hemorrhage

A

may be associated with heparin induced thrombocytopenia

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123
Q

Cd1a + S100 + PDL1+

A

Langerhans Cell Histiocytosis

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124
Q

agranular/hypogranular platelets and impairment to arachidonic acid/thromboxane pathway, + development of myelofibrosis

A

Gray platelet syndrome- NBEAL2 mutation

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125
Q

mechanism of action of luspatercept, use

A

inhibits SMAD signaling, binding and trapping TGFB- promotes late erythropoiesis. Use most for transfusion dependent MDS- works best in MDS + ring sideroblasts

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126
Q

Use of post transplant cyclophosphamide

A

associated with lower incidence of GVHD

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127
Q

CDKN2A mutation

A

dysplastic nevus syndrome, autosomal dom

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128
Q

Cervical cancer risk factors

A

HPV infection, smoking, OCP (prolonged OCP use >5y)

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129
Q

mechanism of HPV genes and tumorogenesis

A

16,18 HPV-> E6 And E7 inactivate Rb1 and p53

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130
Q

HPV vaccine age

A

2 doses at age 11 or 12 (0, 6-12 months later)

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131
Q

Minimally invasive vs radical hysterectomy for early invasive cervical cancer

A

minimally invasive surgery was INFERIOR, radical hysterectomy is standard

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132
Q

Indication for post operative chemoradiation for cervical cancer

A

High Risk= +pelvic nodes, positive surgical margins, positive parametria= CHEMORADS

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133
Q

first line metastatic cervical cancer

A

cisplatin, paclitaxel, + bevacizumab

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134
Q

Adjuvant chemotherapy vs chemoradiation for endometrial cancer

A

modest OS benefit with + radiation, consider mainly for stage III disease AND using chemo+rads for serous type (copy number high)

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135
Q

2nd line treatment for endometrial cancer (non mSI-H)

A

Lenvatinib + Pembrolizumab (ORR 37%)

136
Q

new treatment for epithelioid sarcoma

A

IL1 overxpression-> EZH2, tx- Tazemostat

137
Q

Desmoid tumor treatment

A

Sorafenib for advanced/refractory, some will resolve just with observation

138
Q

Dermatofibrosarcoma Protuberans (DFSP), translocation and treatment

A

t(17,22)- PDGFB, treatment with imatinib

139
Q

Sequence for GIST treatment

A

Imatinib-> Sunitinib-> Regorafenib-> Ripretinib/Avapritinib

140
Q

HPV assoc with cervical squamous vs adeno

A

squamous= 16, adeno= 18

141
Q

Inflammatory breast cancer pearls

A

automatically T4 (T4d), no need for dermal lymphatics on path (clinical dx), use ddACT (neoadjuvant chemo in most cases), may not have discrete mass

142
Q

Treatment of vulvar cancer with 1 mm or less invasion

A

wide local excision

143
Q

BRCA chromosomes

A

BRCA1: chr 17, 50-85% lifetime risk of female breast cancer, 40% ovarian. BRCA2- chr 13

144
Q

Sickle cell disease rule of 3s

A

3 or more pain episodes/year = hydroxyurea
HbS <30% to prevent progression of cerebral ischemia
3 bugs from autosplenectomy (Strep. pneumo, Haemophilus, Meningococcus)

145
Q

VWD

A
  • Type 1: LOW vWF:RCo and LOW vWF:Ag, ratio >0.6
  • Type 2A: LOW FVIII, Very LOW vWF:RCo, LOW vWF:Ag, large multimers absent, normal platelet count and RIPA.
  • Type 2B: Same as 2A + LOW platelet count, HIGH responsiveness to RIPA .
    Type 2N: Very LOW FVIII, ratio of FVIII to vWF:Ag <= 0.6
  • Type 2M: LOW FVIII, LOW vWF:Ag, Very LOW vWF:RCo. All multimers present
146
Q

Indications for LP in AML

A
  • WBC >40-50,000
  • Biphenotypic leukemia
  • Monocytic
  • Extramedullary disease
147
Q

Uterine serous carcinoma- biomarker indication

A

HER2 (30% of uterine serous)- carbo/taxol/trastuzumab

148
Q

Adrenal carcinoma treatment

A

resect! poor prognosis if >5 cm

149
Q

genetic event common in all germ cell tumors and related secondary somatic carcinomas from teratomas

A

12p amplification

150
Q

MDS/AML from teratoma originates from what primary site

A

almost always a mediastinal non seminoma

151
Q

PPI effect on erlotinib

A

decrease absorption (require low PH for absorption)

152
Q

Weight based dosing for obese patients

A

Use actual body weight- exceptions are 2 mg for vincristine, carboplatin (cap to GFR 125-150)

153
Q

lab monitoring with EGFR TKI

A

hypomagnesemia

154
Q

MEK inhibitor side effects

A

Fever (can be hypotensive-fluid unresponsive), rare blurry vision, retinal detachment

155
Q

mtor inhibitor side effects

A

stomatitis, pneumonitis, hyperglycemia, hyperlipidemia (may need statins and metformin)

156
Q

indication for post mastectomy radiation

A

4 or more axilla lymph nodes, also can be considered 1-3 + nodes

157
Q

indications for imaging in breast cancer

A

PET only indicated from inoperable stage III breast cancer

158
Q

HER2+ criteria

A

If IHC 2+, count at least 20 cells with IHC 2+-> HER2/CEP17<2 with >6 HER2 signals per cell= positive

159
Q

most sensitive test for dabigatran

A

thrombin time

160
Q

duration of post operation VTE prophylaxis (ex: colon cancer surgery0

A

5 weeks

161
Q

edoxaban vs LMWH in cancer VTE

A

Noninferior to LMWH in the composite endpoint of recurrent venous thromboembolism (VTE) and major bleeding. However, the risk of recurrent deep venous thrombosis was lower (although not for VTE overall), and the risk of major bleeding was higher with edoxaban.

162
Q

reasons for minor elevation of BHCG

A

hypogonadism, hyperthyroidism, marijuana use

163
Q

Main inheritance pattern for VWD

A

Autosomal dominant

164
Q

elevated PT/PTT and prolonged TT

A

Hypofibrinogenemia or dysfibrinogenemia (vs factor 2, 5, 10 problems will be PT/PTT prolonged but normal thrombin time)

165
Q

large platelets, thrombocytopenia, sensorineural hearing loss, renal failure, and dohle inclusions

A

MYH9 mutation

166
Q

High PTT, bleeding after surgery, high prevalance in Jewish population

A

Factor 11 deficiency

167
Q

Pt with bleeding gets plasma and RBC, then develops fever + dyspnea + pulm infiltrates

A

FFP-> most with TRALI-> mechanism: donor plasma with antibodies to recipient HLA granulocytes

168
Q

hemolytic anemia, swift increase in retics with splenectomy

A

PK deficient (in which retics preferentially sequestered)

169
Q

mutation that predict for aspirin benefit in colon ca

A

PIK3CA

170
Q

INR >10 and no bleeding on warfarin

A

hold warfarin, give oral Vit K, no FFP or PCC

171
Q

possible treatment for radiation necrosis of CNS if cant give steroids

A

Bevacizumab

172
Q

Markers for AML, which ones poorer prognosis

A

CD11+, CD13+, CD33+, POOR RISK: CD34, CD56 (with t8,21 usually)

173
Q

aplastic anemia -which atg

A

HORSE >Rabbit

174
Q

Pneumonias + extensible limbs + eczema + high IgE

A

Hyperimmunoglobulin E, recurrent infection syndrome- 2 ROWS of teeth (baby teeth dont fall), mutations in stat3

175
Q

short stature + cardiomyopathy + neutropenia

A

Barth syndrome

176
Q

hypoplastic marrow, dysplastic nails, pulmonary fibrosis, oral leukoplakia

A

Dyskeratosis Congenita, mutations in DKC1, TERC, TER short telomeres

177
Q

WT1+ calretinin +

A

Mesothelioma

178
Q

Mutations in HLH (primary)

A

PRF1, UNC13D, STX11

179
Q

flower cells leukemia/virus association

A

Adult T cell leukemia /HTLV1

180
Q

epitheliod sarcoma and mutation and treatment

A

EZH2, Tazemetostat, ORR 15%

181
Q

cetuximab reaction, can you try panitumumab?

A

yes- cetux is anti-EGFR chimeric, panitumumab is humanized so can try it

182
Q

Tipraricil mechanism of action

A

inhibits thymidine phosphorylase

183
Q

new liver nodules for a patient on surveillance for colon cancer who got adjuvant FOLFOX 3 years ago

A

always biopsy- can have focal nodular liver lesions, has been associated with oxaliplatin

184
Q

Arsenic and cardiac toxicity

A

blocks K currents, can cause QT prolongation- maintain K and mg

185
Q

KMT2A AML (t v,11)

A

high risk, transplant (MLL-KMT2A)

186
Q

SIckle cell vs Sickle Beta Thal electrophoresis

A

B thal- still have some HbA but not absent

187
Q

anemia 70% HbA and 30% Hb F

A

hereditary presence of Hgb F is a benign condition- mutation in B gene

188
Q

early stage Hodgkin- unfavorable features

A

Bulky mediastinal, >10 cm, B symptoms, ESR >50, >3 sites of disease

189
Q

t(3,14) NHL

A

favorable, BCL6

190
Q

Type 1 vs 3 VWD inheritance

A

Type 1- aut recessive, Type 3- aut dom

191
Q

baby born with thrombocytopenia and develops brusiing and lesions to necrosis

A

congenital protein C deficiency (purpura fulminans)

192
Q

meningococcal septicemia and new brusising and necrotic lesions

A

purpura fulminans due to acquired protein C def

193
Q

Myeloma renal impairment tx

A

bortezomib based tx, can give carfilzomib above cr cl >15

194
Q

platelet receptor involved in platelet adhesion to vessel wall

A

Gp1b/Ix/V

195
Q

Upshaw Schulman disorder

A

rare hereditary deficency of adamts13- congenital TTP

196
Q

thienopyridine associated TTP

A

ex: clopidogrel- can be fast, <2 weeks from exposure

197
Q

after abo and Rh, most common system

A

anti-Kell

198
Q

warts, hypogammaglobulinemia, leukopenia

A

CXCRmutation= WHIM, can tx with plerixafor

199
Q

if come off imatinib with good response CML, at what level do need to reinitatie

A

IS >0.1-> loss of major molecular response

200
Q

Cold agglutinin disease vs PCH

A

Cold Agglutinin: usually underlying monoclonal, (can be polyclonal if viral) IgM, directed against RBC I or i, DAT + anti-C3, EXTRAVASCULAR hemolysis
PCH: polyclonal, children, post viral or post measles vaccine, INTRAVASCULAR hemolysis, IgG directed at P antigen

201
Q

ring sideroblasts -non MDS reasons

A

alcohol, INH, pregnancy, copper def, lead toxicity

202
Q

CLL relapsed options

A

Acalabrutinib, Ibrutinib, Duvelisib, Idelalisib + rituximab

203
Q

Nilotinib cardiac SE

A

prolonged QT interval- check EKG (hold if QTC >480 msec)

204
Q

post transplant consolidation for high risk classical HL

A

brentuximab vedotin

205
Q

amyloidosis poor prognostic translocation

A

11,14

206
Q

risk of thrombosis in CALR vs JAK

A

CALR is lower risk

207
Q

Lymphomatoid Granulomatosis treatment

A

rare EBV related lymphoproliferative neoplasm- lungs nodules +, treatment if high grade is RCHOP with ARA-c

208
Q

treatment of localized small cell of the bladder

A

neoadjuvant cis/etop followed by radiation or cystectomy

209
Q

localized pure adenocarcinoma of the bladder

A

no data for neoadjuvant or adjuvant tx, usually cystectomy

210
Q

risk of progression from MBL to CLL needed treatment

A

about 1-2 %/y

211
Q

Fludarabine considerations in CLL

A

need to give antiviral prophylaxis, risk of secondary MDS/AML, can precipitate hemolytic anemia so dont give if having hemolysis from CLL

212
Q

leucovorin mechanism for working with 5-FU

A

increases inhibition by 5FU inhibiting thymidilate synthase

213
Q

MYH + surveillance

A

colonoscopy at age 25-30, consider EGD, yearly testicular US

214
Q

BRCA1 surveillance

A

start annual breast MRI by age 25. for age 30-75: annyal mammo and breast MR

215
Q

localized Merkel cell treatment

A

wide local excision + SLNB

216
Q

Polatuzumab target

A

refractory DLBCL, anti-CD79b (B cell receptor)

217
Q

features of CALR ET

A

higher plt count, concern for VWF antigen, esp if factor activity level <30%

218
Q

first line treatment of amyloidosis

A

Dara-CyborD

219
Q

Hypoplasia definition after AML induction, when to reassess

A

if cellularity <20% which blasts <5%-> await recovery, repeat bone marrow biopsy

220
Q

CML type with marked neutrophilia

A

BCR ABL p230

221
Q

when is CNS prophylaxis given for APL? Type of transplant?

A

after second remission, then proceed to autologous transplant

222
Q

Hexagonal crystals and anemia

A

HbC- beta globin mutation, glutamic acid-> lysine

223
Q

indications and duration of anticoagulation for LE superficial DVT

A

higher risk if >5 cm, close to DVT (esp saphenofemoral or saphenopoliteal)- anticoagulation for 45 days

224
Q

cirrhosis + incidental portal vein thrombosis management

A

can observe with cirrhosis- no anticoagulation. consider if symptomatic, and also if extending into mesenteric vein

225
Q

unprovoked left iliofemoral DVT in woman age 30

A

check for May thurner- contrast venogram

226
Q

Pomalidomide can be given with renal failure

A

yes- with dose changes

227
Q

how to prevent complications of neonatal hemochromatosis with mom with disease

A

weekly IVIG in pregnancy

228
Q

quinine mechanism for thrombocytopenia

A

neoantigen formed binds to Gp 2b/3a

229
Q

which antibiotic has anti-amyloidosis properties

A

doxycycline

230
Q

APL high risk and treatment

A

WBC >10- ATRA+Dauno or Idarubicin+ Arsenic )may have cytrabine or gemtuzumab)

231
Q

MDS after 1 y of chemo- which one

A

Topo inhibitors= doxorubicin, rearrangements in 11q23

232
Q

when is ibrutinib stopped prior to surgery

A

3-7 days

233
Q

early localized Hodgkin unfavorable treatment

A

ABVD x 4 + ISRT (RT improves EFS but not OS)

234
Q

Erdheim chester BRAF + treatment

A

Vemurafenib

235
Q

DRVVT starts with affecting what factor

A

Factor X

236
Q

child with prolonged bleeding after procedure, normal coags, fibrinogen, platelet function

A

a2-antiplasmin deficiency

237
Q

17p deletion and CLL treatment- what can you not give?

A

any chemo-immunotherapy- no FCR/chlorambucil based regimen

238
Q

best prognosis CLL

A

13q del

239
Q

T cell malignancy associated with rheumatoid arthritis

A

T-cell LGL

240
Q

how long of remission ideal to retry same agent (ex: cladribine) with hairy cell

A

2 y

241
Q

mutation leading to ibrutinib resistance

A

C481 on BTK

242
Q

BPDCN immunophenotype

A

CD4+ CD56+ CD123+

243
Q

treatment of B cell prolymphocytic leukemia

A

ibrutinib

244
Q

Grade 3 B Follicular lymphoma

A

> 15 centroblasts/hpf + solid sheets of centroblasts, tx with RCHOP, even if asx. Likely to be CD10- BCL2- and lack t14,18 unlike other FL

245
Q

antibiotic resistant translocation-h.pylori malt lymphoma

A

t(11,18)

246
Q

lymphomatous polyposis associated with

A

mantle cell lymphoma

247
Q

primary effusion lymphoma

A

HIV associated, etiology is hHV 8

248
Q

S100+ large LN

A

Rosai Dorfman- can observe if asx

249
Q

PFS for initial RVD, transplant and len maintenance

A

50 months

250
Q

limitation of pluripotent stem cell for gene therapy

A

genomic instability, and pluripotent stem cell derived tissue may be immunogenic

251
Q

localized vs systemic treatment with POEMS syndrome

A

localized radiation can be curative if 2 plasmacytomas or lyic bone lesions, no clonal cells in marrow. otherwise can do systemic therapy- auto transplant reasonable

252
Q

serious fungal infection associated with ibrutinib tx

A

invasive aspergillosis

253
Q

12 days after transplant- fever, dyspnea, lung consolidation, macular rash with thrombocytopenia

A

Engrafment syndrome, tx with steroids

254
Q

bone marrow vs blood transplant source difference

A

bone marrow: higher graft failure, longer time to engrafement= higher risk of infection
blood: higher incidence of GVHD

255
Q

SOS after transplant

A

hepatomegaly, can have renal failure-> defibrotide

256
Q

MDS development after aplastic anemia- cytogenetic change

A

monosomy 7

257
Q

management of isolated CNS relapse of ALL

A

aggressive tx with cranial irradiation, Intra ventricle chemo and re-induce for ALL

258
Q

pre-B cell ALL with eosinophilia- translocation

A

t (5,14) (q31,32)-> IL3-IgH

259
Q

B-all poor prognostic factor

A

IKAROS

260
Q

mechanism of iron overload with ineffective erythropoiesis in MDS

A

increased erthroferrone made from erythrobalsts-> decrease hepcidin, and increased iron absorption

261
Q

Deferasirox monitorihng when starting

A

weekly renal monitoring

262
Q

treatment of mild porphyria

A

glucose loading, IV fluids, pain control

263
Q

Accelerated Phase CML

A

look for 20% >basophils and plt <100,000. Blast -phase is usually 20% blasts

264
Q

When is FCR contraindicated in CLL

A

if have 17p del/TP53, or also if already has autoimmune hemolytic anemia

265
Q

cytogenetic abnormality poor adverse risk for amyloidosis

A

t(11,14)-> ongoing trials with venetoclax

266
Q

PRRT long term risk

A

AML/MDS

267
Q

hypoplastic MDS markers and treatment

A

HLADR2 and DR15, treat like aplastic anemia- cyclosporine/ATG

268
Q

2 major criteria for POEMS

A

polyneuropathy + monoclonal plasma cell disorder

269
Q

adjuvant bisphosphonate in breast cancer

A

improved survival in postmenopausal non metastatic breast cancer

270
Q

Relapsed Kaposi Treatment

A

Pomalidomide (others- bevacizumab, etoposide, imatinib)

271
Q

molecular testing for anaplastic thyroid cancers

A

BRAF V600 (can use dab/tram)

272
Q

cancer drugs that can cause TTP/HUS

A

Gemcitabine, Mitomycin

273
Q

breast cancer chemo safe in 2nd trimester

A

FAC (5-Fu, adriamycin, cyclophosphamide)

274
Q

Paraneoplastic lung cancer syndromes- name the antibody: Cerebellar degneration

A

Anti-Hu

275
Q

Paraneoplastic lung cancer syndromes- name the antibody: Opsoclonus/myoclonus

A

Anti-Ri

276
Q

Paraneoplastic lung cancer syndromes- name the antibody: Stiff person

A

Anti-amphiphysin

277
Q

adjuvant tx of uterine carcinosarcoma

A

chemotherapy + EBRT +/- vaginal brachytherapy

278
Q

diffuse alveolar hemorrhage-% of hemosiderin laden macrophages for dx

A

20%

279
Q

Selenixor mechanism of action

A

XPO1 inhibitor

280
Q

Nivo vs Pembro indications in 2nd line esophageal cancer

A

Nivo for any esophageal squamous. Pembro for 2nd line esophageal squamous with CPS 10

281
Q

mutation in mesothelioma

A

BAP1

282
Q

what to do if at 3months of CML tx BCR AbL 25%

A

should be <10%, but if more than 50% reduction compared to baseline or minimally above 10% cutoff-can continue same dose of 2nd gen TKI, but should increase imatinib dose

283
Q

Tx of MPN 5q33

A

Imatinib (encordes PDGFR)

284
Q

papillary thyroid cancer- need for completion thyroidectomy when?

A

+ margins, + LN, +vascular invasion, + tumor size >4

285
Q

carcinoid tumors of appendix- when do you need a right hemicolectomy?

A

> 2 cm, + margins, perforated appendix

286
Q

Number of LN evaluate for gastric cancer

A

15

287
Q

neutropenia with ABVD and hodgkin

A

continue WITHOUT growth factor, low incidence of febrile neutropenia, growth factors may increase bleo pulm toxicity

288
Q

medullary breast cancer management

A

higher nuclear grade, but treat like ductal cancer, may have more favorable prognosis

289
Q

Dose for 7+3 AML induction

A

Cytarabine 100-200 mg/m2 x 7 days and either Idarubicin 12mg/m2 or Dauno 60-90 mg/m2 x3 days

290
Q

when to check bone marrow in hairy cell

A

if having hematologic recovery and spleen size reduction, dont check before 4 months after tx

291
Q

When to use imatinib adjuvant for GIST

A

No imatinib benefit if no KIT or PDGFRA mutations, also if PDGFRA D842V
Give if >5 cm and 5mitoses, or >10 cm OR >10 mitoses OR rupture- for 3 years

292
Q

for her 2 and TNBC when should adjuvant tx be by

A

8 weeks

293
Q

RAI adjuvant doses for low risk disease (tumor <4 cm, N0 disease)

A

30 mCi (higher doses are 100-200 mCI)

294
Q

Vitamin E and prostate cancer

A

increased risk

295
Q

80% of merkel cell with what virus

A

polyomavirus

296
Q

Indication for total thyroidectomy for papillary thyroid cancer

A

> 4 cm with central neck nodes

297
Q

what agents cannot be used with prior seizure hx in prostate cancer

A

enzalutamide and apalutumide

298
Q

eye exam standard of care dx for CNS lymphoma

A

Slit lamp + fundoscopic exam

299
Q

HPV risk factors for cervical cancer

A

mainly 16,1 8 HPV 16 and 18 are responsible for 70% of all cervical cancers, but HPV 58 and 33 are also associated with cervical cancer (~10%) and represent a risk factor

300
Q

what decreases severity of mucositis with bolus 5-FU

A

Oral cryotherapy can decrease the severity of bolus 5-FU associated mucositis

301
Q

In what situation has rituximab shown to improve OS in lymphomas

A

2 cases in mantle cell

  • elderly pt after RCHOP
  • after induction DHAP + auto
302
Q

if GBM and already on keppra 1000 mg BID and dex 4 mg BID and have seizures again- next step

A

need to expedite surgical resection

303
Q

recurrent desmoid tumor treatment

A

sorafenib

304
Q

association of renal cancer and sickle trait- which kind

A

renal medullary carcinoma, +hemorrhage and necrosis, +AE1, AE3, CEA

305
Q

tubular breast cancer prognosis

A

usually excellent, ER+, good overall response with surgical removal alone, do adjuvant endocrine therapy like usual

306
Q

mutation with granulosa cell tumors of ovary

A

FOXL2

307
Q

if ALK FISH +, do need to wait for FISH?

A

no

308
Q

There is no proven survival benefit for early initiation of ADT, particularly for slow PSADT or Gleason 7 and absence of metastatic disease. T or F?

A

true

309
Q

risk factors for breast angiosarcoma

A

prior ionizing radiation, chronic lymphedema

310
Q

Fibromellar carcinoma

A

rare hepatic tumor- not related to cirrhosis or Hepaitits, well circumscribed hepatic mass. will get biopsy with DNAJB1-KRTKACA fusions

311
Q

DES exposure and risk of what type of vaginal cancer

A

adenocarcinoma (NOT squamous)

312
Q

Vincristine max

A

2 mg

313
Q

Use of bisphosphonate in adjuvant breast cancer

A

q6 months for postmenopausal women- decrease recurrent, improvement seen in mortality!

314
Q

bleomycin and skin toxicity

A

flagellar erythema

315
Q

bleomycin relative contraindications

A

age >50, any lung disease, reduced renal function

316
Q

spermatocytic seminomas

A

usually older patients, no relation to cryptorchidism, no elevated markers. Orchiectomy usually sufficient

317
Q

MRI brain indication for germ cell tumors

A

Bhcg >5000

318
Q

Infusional 5FU vs Bolus

A

Infusional= lesser myelosuppression, more hand-foot syndrome, CORONARY VASOSPASM, slight advantage in tumor response

319
Q

paclitaxel toxicity

A

bradycardia, AV nodal blockade

320
Q

sudden arrthymia after starting AML therapy

A

can have sudden hypokalemia associatedw ith acute leukemia

321
Q

Letermovir use

A

inhibits CMV teminase complex, used in CMV + transplant recipients

322
Q

genetic GIST mutation

A

SDH

323
Q

Gorlin syndrome

A

mandibular tumors, medulloblastomas. Mutations in PTCH1 or SUFU (hedgehog pathway)

324
Q

CHEK2 cancer risks

A

breast, colon

325
Q

PSA screening for BRCA2

A

age 40

326
Q

HOXb13 and development of which cancer

A

familial association -prostate

327
Q

if starting ciprofloxacin, what should do with ibrutinib

A

DECREASE DOSE! as cipro (quinolone) is an CYP inhibitor= so will increase Ibrutinib level

328
Q

drugs that displace methotrexate and can increase toxicity

A

salicylates, ibuprofen, sulfa, phenytoin

329
Q

chemo that requires seizure ppx

A

Busulfan

330
Q

Pt getting FOLFIRI- neutropenia

A

UGT1A1 status for irinotecan

331
Q

TPMT genotype deficient

A

TPMT breakds down 6MP. if lack= lower 6MMP (methylMP), higher level of thioguanine nucleotides. If homozygous- dose reduce 6MP by 80-90%, if heterozygous -10-15%

332
Q

Allopurinol and 6-MP interaction

A

Allopurinol inhibit XO. 6MP catabolized by XO. if both tgether- then risk of 6-MP toxicity. Need to reduce dose of 6MP by 50%

333
Q

what lab to monitor closely with cetuximab or panitumumab

A

Magnesium

334
Q

Lapatinib rash

A

looks like acne with papules and pustules- HOLD drug, use oatmeal cream, clindamycin. NOT RETINOID cream

335
Q

Aspariginase and MTX dosing together?

A

Asparaginase can rescue from MTX toxicity-> so give MTX and THEN asparaginase

336
Q

intraductal papilloma of breast

A

surgical excision- is benign, but can be large, +blood ischarger. Can copresent though with premalignant features. Do not metastasize.