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Hematology/Medical Oncology ABIM > Hematology/Medical Oncology > Flashcards

Hematology/Medical Oncology Flashcards

ABIM Board Review

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USMLE® Step 1 flashcards
1
Q

Adjuvant chemotherapy in pancreatic cancer s/p R1 resection?

A

Gemcitabine/CIsplatin

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2
Q

Least immunogenic form of L-asparginase?

A

Pegaspargase • Up to 1/3 of pts who do not have a symptomatic reaction can develop IgG Abs against L-asparginase that can reduce its efficacy

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3
Q

CD5+, CD23+, dim CD20, CD10(-), cyclin D1(-)

A

CLL Immunophenotype

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4
Q

Treatment of Low-Risk Gestational Trophoblastic Disease

A

Either single-agent MTX or Dactinomycin

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5
Q

Management of early stage HCC s/p complete surgical resection with negative margins?

A

Surveillance only • Currently there are NO approved adjuvant therapies for HCC

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6
Q

+CD2, +CD3, +CD4, +CD5, +CD25 (bright)

A

ATLL Immunophenotype

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7
Q

+CD4, CD7-, CD25-

A

T-cell Prolymphocytic Leukemia Immunophenotype

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8
Q

+CD4, CD7-, CD25-

A

Mycoses Fungicides/Sezary Syndrome Immunophenotype

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9
Q

5-Azacytadine MOA?

A

5-azacytadine is a cytosine nucleotide that is incorporated into DNA and inhibits DNA methylation, resulting in reactivation of tumor suppressor genes that were previously silenced through methylation

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10
Q

What is the Platelet Defect & Clinical Presentation in Hermansky-Pudlak Syndrome?

A

Hermansky-Pudlak Syndrome: - MOA: Absence of Dense Plt Granules (Plt storage pool defect) • Autosomal Recessive mutations in HPS-1 (most common), DTNBP1, AP3B1 genes - Clinically p/w Oculocutaneous Albinism, Congenital Nystagmus, vision problems, granulomatous colitis, pulmonary fibrosis - More common in Puerto Rico - Diagnosis made with absence of dense granules on electron microscopy - Plt Function Studies: Reversal of platelet ADP:ATP ratio is diagnostic but not specific for this disorder

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11
Q

RAI Staging in CLL

A

RAI STAGE: 0 = Lymphocytosis in blood/BM 1 = Lymphocytosis + LAD 2 = Lymphocytosis + SM 3 = Lymphocytosis + Hb<11 4 = Lymphocytosis + Plt<100K

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12
Q

Identify the Low Risk and High Risk (for recurrence) pathologies in Early-Stage Ovarian Cancer and describe how is each managed

A

Early-Stage Ovarian Cancer • Low-Risk: Serous carcinoma • High-Risk: All other pathologies (i.e. mucinous carcinoma, clear-cell carcinoma) require adjuvant platinum-based chemotherapy

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13
Q

First and Second Line Therapies for Advanced/Metastatic Cholangiocarcinoma

A

1st Line: Cisplatin/Gemcitabine 2nd Line: FOLFOX (ABC-06 study)

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14
Q

Hormone-receptor status in Type 2 Endometrial Cancer

A

• Type 2 Endometrial carcinoma is not associated with estrogen exposure and the tumor is usually negative for ER and PR • Mutations in Type 2 endometrial cancer involve p53, HER2 amplification, BCL-2 amplification

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15
Q

MYD88 L265 is present in which hematologic malignancy?

A

Molecular testing for MYD88 L265 is used to differentiate Waldenstrom Macroglobulinemia from multiple myeloma

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16
Q

Poor Prognostic Indicators in Hodgkin’s Disease

A
  1. Age>50 2. Bulky Disease (LAD>7cm) 3. ESR>30 with +B-symptoms ESR>50 w/o B-symptoms 4. Involvement of 4 or more lymph nodes 5. Mixed-cell histology
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17
Q

Treatment of metastatic GIST

A

Imatinib 400mg QD - Median time to see clinical response to therapy is ~4 months and maximum response seen at ~6 months (GIST: Spindle-cell neoplasm, CD117+, CD34+)

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18
Q

What is the platelet defect in Glanzmann’s Thrombasthenia and how do you treat bleeding in these patients?

A

Glanzmann’s thrombasthenia is a rare coagulopathy in which the platelets contain defective or low levels of glycoprotein IIb/IIIa (GpIIb/IIIa) which is a receptor for fibrinogen. As a result, no fibrinogen bridging of platelets to other platelets can occur, and the bleeding time is significantly prolonged. PLT FUNCTION STUDIES: +Ristocetin and absent response to all other agents TREATMENT: Plt transfusions and recombinant Factor VIIa (Novoseven)

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19
Q

Treatment for Metastatic Head and Neck SCC?

A

5FU+Cisplatin+Cetuximab

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20
Q

Management of MGMT methylation-negative Glioblastoma s/p subtotal resection in elderly patients (>70yoa)?

A

RT Monotherapy (40Gy in 15fx)

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21
Q

What is the defect in Type 2N VWD and how is it diagnosed?

A

Type 2N VWD is due to defective binding of VWF to F8 . - Female pts may present with hemophilia-like bleeding symptoms - LABS: low VWF, low F8 and VWF:F8 binding assay would show absence of VWF binding to F8

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22
Q

Germ Cell Tumors

A
  • Seminomas and teratomas often p/w normal tumor markers. Seminomas usually are diagnosed in early stages. - Yolk sac tumors: associated with elevated AFP levels and frequently a component of mixed non-seminomatous GCTs - Choriocarcinomas: typically p/w very elevated serum hCG levels, widespread hematogenous metastases and tumor hemorrhage
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23
Q

Management of Early-Stage Cervical Cancer

A

Stage IA1: cervical conation or total (type 1) hysterectomy - If pathology from conation is negative for LVI, no adjuvant therapy is indicated. Stage IB1: Tumors>7mm but <4cm in horizontal spread - Radical surgery with LN dissection indicated if deep stromal invasion or +LVI is present Treatment of more advanced stages of cervical cancer (Stage IIA and higher) Involve systemic cisplatin-based chemo and vaginal brachytherapy

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24
Q

Identify the adverse risk pathology features in resected oral cancer

A
  1. T stage>2 2. Node-positive disease 3. +Extracapsular nodal spread/extension 4. Positive surgical margins 5. +PNI 6. +LVI
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25
Q

ETV6-NTRK3 gene fusions is pathognomonic for which malignancy?

A

Mammary analogue secretory carcinoma (MASC)

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26
Q

Li-Fraumeni Syndrome

A

Autosomal Dominant familial cancer d/o involving germline TP53 mutation - Breast cancer is the most common type of cancer in Li-Fraumeni pts with 30% of them diagnosed <30yoa

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27
Q

Which EGFR Inhibitor is effective against NSCLC tumors containing the L858R mutation (Exons 19 or 21)

A

Afatinib

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28
Q

First-line therapy for Kaposi’s Sarcoma

A

Doxil (Liposomal Doxorubicin)

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29
Q

What is recommended for surveillance management in patients with Lynch Syndrome?

A
  1. Prophylactic hysterectomy & bilateral salpingoopheretomy (TAH BSO) due to 40% lifetime risk for endometrial cancer in females with Lynch Syndrome 2. Annual U/A (increased risk for urothelial cancer) 3. Screening upper and lower GI endoscopies Q3-5 years (increased risk for upper GI malignancies) 4. Skin exam (skin cancer) • Pts are also at increased risk for pancreatic cancer
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30
Q

What is the treatment for neurotoxicity from high-dose ifosfamide?

A

Methylene blue - Neurotoxicity from ifosfamide can p/w delirium, aphasia, ataxia and lethargy. Onset can be acute and clinical presentation can be similar to CVA.

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31
Q

What is the platelet defect in Bernard Soulier Syndrome and what are the clinical manifestations?

A

Decreased GPIb/V/IX on platelets - p/w thrombocytopenia and giant platelets - Platelet function studies show abnormal absence of response to ristocetin; all other results normal (Opposite to Glanzmann’s thrombasthenia: +response to ristocetin and absent response to all other agents)

32
Q

Treatment of Post-Transfusion Purpura

A

High-dose IVIG (2mg/kg) given over 2-5 days - If refractory to IVIG, consider plasma-exchange for 2nd-line therapy

33
Q

Birt-Hogg Dube Syndrome (BHD)

A

Familial Cancer Syndrome - Autosomal Dominant germline mutation in Folliculin gene located on the short arm of Chromosome 17 - Characterized by: 1. benign hair follicle tumors (fibrofolliculoma) 2. pulmonary cysts 3. spontaneous PTX 4. 7X increased risk for RCC (30% of pts; chromophobe renal carcinoma and hybrid oncocytic tumors most common subtypes)

34
Q

CD79a

A

CD79a is a B-cell marker, specifically it is a B-cell antigen receptor complex-associated protein alpha chain and MB-1 membrane glycoprotein

35
Q

F7 deficiency

A
  • Elevated PT and normal PTT on labs accompanied clinically by s/sxs of mucocutaneous bleeding of varying severity - Treatment: recombinant FVIIa, other F7 concentrate or PCC
36
Q

What is the strongest predictor for treatment response in anaplastic oligodendroglioma?

A

Presence of BOTH del 1p and del 19q is associated with improved OS

37
Q

What are the preventive/screening tests recommended for BRCA1/2 positive patients?

A

Females: • Breast MRI starting at age 25 THEN • Starting age 30: Alternate between Breast MRI and Mammogram Q6months • Risk-reducing salpingo-oopherectomy (RRSO) starting 35-40yo upon completion of childbearing • In BRCA1 positive women only: recommend prophylactic risk reducing TAH-BSO due to high risk of serous ovarian cancer which is difficult to diagnose • If RRSO not elected, transvaginal US combined with serum CA-125 although benefit is uncertain Males: • Prostate ca screening starting at age 45 • Annual clinical breast exam

38
Q

Identify Familial CRC Syndromes that involve mutations in tumor suppressor genes

A
  1. Li-Fraumeni: TP53 2. FAP: APC 3. Peutz-Jeghers: STK11 4. Cowden: PTEN
39
Q

What are the genes implicated in Lynch Syndrome (aka HBPCC)?

A

MLH1, MSH2, MSH6, PMS2, EPCAM - Autosomal Dominant - Standard of care to screen all colon and uterine cancers for MSI

40
Q

MEN2A

A

RET Mutation Hyperparathyroidism Medullary Thyroid cancer Pheochromocytoma

41
Q

Identify the genetic mutation and clinical features of Cowden Syndrome.

A

PTEN mutation ***Macrocephaly ***Papillary or Follicular Thyroid Cancer - GI polyps (hamartomas) - Cutaneous lesions, oral papillomas - Also asst’d with increased risk for Breast, Uterine and Kidney cancers

42
Q

MDS IPSS-R

A
  1. BM Blast% <=2% = 0 point >2<5% = 1 point 5-10% = 2 points >10% = 3 points 2. Cytogenetics: - Very Good Risk (0 points): del(11q), -Y - Good Risk (1 point): normal, del(5q), del(20q), del(12p), double including del(5q) - Intermediate Risk (2 points): +8, del(7q), i(17q), +19, any other single or double anomaly not listed - Poor Risk (3 points): -7, inv(3)/t(3q)/del(3q), double including -7/del(7q), complex: 3 abnormalities - Very Poor Risk (4 points): Complex: >3 abnormalities 3. Hemoglobin: =>10 (0 points) 8 to <10 (1 point) <8 (1.5 points) 4. Plts =>100 (0 points) 50 to <100 (0.5 points) <50 (1 point) 5. ANC =>800 (0 points) <800 (0.5 points) Overall Score: IPSS Subgroup Very Low: <=1.5 Low: >1.5-3 Intermediate: >3-4.5 High: >4.5-6 Very High: >6
43
Q

Genetic Abnormalities Definitive for AML Regardless of Blast Count

A

t(8;21)(q22;q22): RUNX1-RUNX1T1 inv(16)(p13; 1q22) or t(16;16)(p13.1;q22): CBFB-MYH11 t(15;17)(q22;21): PML-RARA

44
Q

Mechanisms of acquired resistance to Irinotecan

A
  1. Increased expression of p170 glycoprotein resulting in increased drug efflux resulting in decreased intracellular accumulation of the drug 2. Mutations in Topoisomerase I enzyme resulting in decreased drug affinity 3. Decreased expression of target Topo I enzyme 4. Changes in irinotecan metabolism resulting in decreased production of its active metabolite SN-38
45
Q

What is the adjuvant treatment for locally advanced high-risk prostate cancer

A

ADT for 18-24 months

46
Q

AML Cytogenetics Risk Stratification

A

Favorable: inv(16) or t(16;16), t(8;21), t(15;17) - (Molecular abnormalities): Normal cytogenetics: WITH isolated NPM1 or CEBPA mutation in the absence of FLT3-ITD Intermediate: Normal cytogenetics, exon 8 trisomy, t(9;11) - t(8;21), inv(16), t(16;16): WITH c-kit mutation Unfavorable: Complex karyotype, -5, 5q-, -7, 7q-, -11q23, t(6;9), t(9;22) - Normal cytogenetics: WITH FLT3-ITD, inv(3), t(3;3)

47
Q

AML Poor-Risk Cytogenetics

A

Unfavorable: Complex karyotype, -5, 5q-, -7, 7q-, -11q23, t(6;9), t(9;22) - Normal cytogenetics: WITH FLT3-ITD, inv(3), t(3;3)

48
Q

AML Favorable-Risk Cytogenetics

A

inv(16) or t(16;16), t(8;21), t(15;17) - (Molecular abnormalities): Normal cytogenetics: WITH isolated NPM1 or CEBPA mutation in the absence of FLT3-ITD

49
Q

Hereditary TTP

A

Autosomal Recessive severe deficiency in ADAMTS13 without an inhibitor - patients p/w microangiopathic hemolytic anemia (MAHA) in neonatal `period that resolve with plasma infusion

50
Q

Treatment for recurrent/refractory advanced medullary thyroid cancer

A

Cabozantinib OR Vandetinib • Both have been shown to significantly prolong PFS in r/r advanced MTC

51
Q

What is the most common underlying cause of GVHD?

A

MHC incompatibility between donor and recipient. - Interaction between donor and host cells leads to possible activation and clonal expansion of donor T lymphocytes followed by cytokine release and recruitment of secondary effector cells leading to destruction of the transplant recipient’s cells/tissue

52
Q

Treatment of Multiple Myeloma with CNS involvement

A

Pomalidomide has significant CNS penetration (Pomalidomide/Carfilzomib/Dex regimen has demonstrated good ORR in relapsed MM)

53
Q

Metastatic CSPC

A

ADT (with GnRH antagonist) +docetaxel

54
Q

FDA-approved treatment specifically for therapy-related AML (tAML)

A

CPX-351 - CPX-351 is a dual-drug liposomal encapsulation of cytarabine and daunorubicin that delivers a synergistic 5:1 drug ratio into leukemia cells and shown to improve OS compared to standard 7+3 (cytarabine/daunorubicin)

55
Q

FDA-approved treatment specifically for IDH2 mutant AML

A

Enasidenib

56
Q

Gemtuzumab-Ozogamicin

A

FDA-approved as an additional component of induction regimens for the treatment of adult AML that have strong positivity for CD33+ on IHC

57
Q

Identify the mutation implicated in Grey Platelet Syndrome and its clinical presentation

A

Autosomal Recessive Plt storage pool disease due to - Mutation in NBEAL2 (a vesicle trafficking gene) - Results in absence of platelet alpha-granules (PF4, beta-TG, VWF, fibronectin, F5, PDGFR) - Clinically, pts p/w bleeding diathesis, mild TP, prolonged bleeding time and NORMAL platelet aggregation study results

58
Q

Prognostic Mutations in Glioblastoma

A

FAVORABLE PROGNOSIS: 1. MGMT promotor methylation 2. IDH-mutant 3. 1p/19q co-deletion UNFAVORABLE PROGNOSIS: 1. +TERT-mutant

59
Q

Atypical HUS

A

aHUS is a TMA due to uncontrolled activation of the alternative complement pathway and clinically p/w: 1. Combs-negative MAH 2. Thrombocytopenia 3. AKI (Note: Typical HUS is associated with E.coli O157:H7 infection)

60
Q

Binemetinib

A

MEK inhibitor shown to have activity in metastatic melanoma with NRAS mutations and BRAF mutations other than the more commonly encountered BRAF V600E and V600K

61
Q

Complete Hyadiform Mole (CHM)

A

CHMs are diploid and either 46XX or 46XY with all chromosomes from paternal origin, resulting from fertilization of an empty ovum by a haploid sperm which then undergoes duplication. - Partial hyadiform moles (PHM) contain bother maternal and paternal chromosomes and typically triploid (i.e. 69XXY), a result of two sperm fertilizing one ovum - CHMs have higher risk of malignant transformation to invasive moles than PHMs

62
Q

Adjuvant therapy for Low-Grade Gliomas s/p resection

A

RT followed by PCV (procarbazine, CCNU and vincristine)

63
Q

Identify the diagnosis for the following patient: 65yoM p/w B-symptoms, generalized LAD, pruritis, rash and HPSM. Lymph node biopsy contained cells with the following immunophenotype: CD4+, CD10+, CD30 dim, sCD7 dim, cCXCL13+, PD1+, ICOS+, sCXCR5+, CD154+, BCL6+. Negative for CD20, Pax-5, Cyclin D1.

A

Angioimmunoblastic T-cell Lymphoma

64
Q

1st-line treatment for Recurrent/Metastatic BOT SCC with PD-L1 CPS 1%

A

Pembrolizumab +cisplatin or carbo+5FU

65
Q

Dexrazoxane

A

Used for primary prevention against anthracycline-induced myocardial toxicity

66
Q

Morphological abnormality seen on peripheral blood smear in patients with Bernard Soulier syndrome

A

Giant Platelets and thrombocytopenia

67
Q

RCC Clinical RIsk Stratification

A

Poor prognostic factors: 1. Low KPS 2. LDH>ULN 3. Hb<10 4. Elevated corrected serum Ca 5. Time from initial RCC diagnosis to start of therapy<1 year • Poor Risk = Presence of 3 or more of the above - Recommended Tx: Ipi/Nivo (if not available, cabozantinib is preferred over sunitinib and pazopanib) • Treatment of Good Risk patients: Anti-angiogenics (sunitinib, pazopanib, cabozantinib) are recommended for 1st-line therapy

68
Q

Treatments for metastatic gastric GIST

A

1st-line: Imatinib 2nd line: Sunitinib 3rd line: Regorafenib

69
Q

54yoM with CKD and anaplastic thyroid carcinoma is receiving Epo for anemia and subsequently develops severe anemia (Hb 6.7). What is the next best step?

A

Discontinue Epo and test for anti-Epo antibodies to r/o pure red aplasia 2/2 anti-Epo Abs

70
Q

Neuroendocrine Tumor Pathology Grading

A
71
Q

WHO Classification of Pancreatic Neuroendocrine Tumors (pNETs)

A
72
Q

Management of Carcinoid Tumor of the Appendix

A
  • Right Hemicolectomy should be considered for tumors>2cm
  • Tumors between 1-2cm with meso-appendiceal involvement may also have +LN involvement but have very low recurrence rates despite this
73
Q

Systemic Treatment Options for Advanced NETs (pNETs vs. Carcinoid)

A
74
Q

Both auto-SCT and allo-SCT recipients are at increased risk for which chronic diseases?

A
  1. CVD
  2. Renal Disease
  3. Diabetes
75
Q

Management of patient with metastatic prostate cancer with bone-only mets currently treated with Lupron+Bicalutamide p/w slowly rising PSA and remains asymptomatic?

A

Discontinue Bicalutamide

  • Discontinue Bicalutamide and monitor for response to anti androgen withdrawal which occurs in ~15-20% of patients with treatment response seen after 3-6 weeks with decline in PSA levels.
  • • Radium-223 is indicated only for patients with symptomatic bone metastases
76
Q

GPII/III Inhibitors (abciximab, eptifibitide, tirofiban) mimic which congenital platelet disorder?

A

Hematology/Medical Oncology ABIM (2 decks)

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