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

Hematology/Medical Oncology Flashcards

ABIM Board Review

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
ETV6-NTRK3 gene fusions is pathognomonic for which malignancy?
Mammary analogue secretory carcinoma (MASC)
26
Li-Fraumeni Syndrome
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
27
Which EGFR Inhibitor is effective against NSCLC tumors containing the L858R mutation (Exons 19 or 21)
Afatinib
28
First-line therapy for Kaposi’s Sarcoma
Doxil (Liposomal Doxorubicin)
29
What is recommended for surveillance management in patients with Lynch Syndrome?
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
30
What is the treatment for neurotoxicity from high-dose ifosfamide?
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.
31
What is the platelet defect in Bernard Soulier Syndrome and what are the clinical manifestations?
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
Treatment of Post-Transfusion Purpura
High-dose IVIG (2mg/kg) given over 2-5 days - If refractory to IVIG, consider plasma-exchange for 2nd-line therapy
33
Birt-Hogg Dube Syndrome (BHD)
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
CD79a
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
F7 deficiency
- 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
What is the strongest predictor for treatment response in anaplastic oligodendroglioma?
Presence of BOTH del 1p and del 19q is associated with improved OS
37
What are the preventive/screening tests recommended for BRCA1/2 positive patients?
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
Identify Familial CRC Syndromes that involve mutations in tumor suppressor genes
1. Li-Fraumeni: TP53 2. FAP: APC 3. Peutz-Jeghers: STK11 4. Cowden: PTEN
39
What are the genes implicated in Lynch Syndrome (aka HBPCC)?
MLH1, MSH2, MSH6, PMS2, EPCAM - Autosomal Dominant - Standard of care to screen all colon and uterine cancers for MSI
40
MEN2A
RET Mutation Hyperparathyroidism Medullary Thyroid cancer Pheochromocytoma
41
Identify the genetic mutation and clinical features of Cowden Syndrome.
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
MDS IPSS-R
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
Genetic Abnormalities Definitive for AML Regardless of Blast Count
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
Mechanisms of acquired resistance to Irinotecan
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
What is the adjuvant treatment for locally advanced high-risk prostate cancer
ADT for 18-24 months
46
AML Cytogenetics Risk Stratification
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
AML Poor-Risk Cytogenetics
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
AML Favorable-Risk Cytogenetics
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
Hereditary TTP
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
Treatment for recurrent/refractory advanced medullary thyroid cancer
Cabozantinib OR Vandetinib • Both have been shown to significantly prolong PFS in r/r advanced MTC
51
What is the most common underlying cause of GVHD?
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
Treatment of Multiple Myeloma with CNS involvement
Pomalidomide has significant CNS penetration (Pomalidomide/Carfilzomib/Dex regimen has demonstrated good ORR in relapsed MM)
53
Metastatic CSPC
ADT (with GnRH antagonist) +docetaxel
54
FDA-approved treatment specifically for therapy-related AML (tAML)
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
FDA-approved treatment specifically for IDH2 mutant AML
Enasidenib
56
Gemtuzumab-Ozogamicin
FDA-approved as an additional component of induction regimens for the treatment of adult AML that have strong positivity for CD33+ on IHC
57
Identify the mutation implicated in Grey Platelet Syndrome and its clinical presentation
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
Prognostic Mutations in Glioblastoma
FAVORABLE PROGNOSIS: 1. MGMT promotor methylation 2. IDH-mutant 3. 1p/19q co-deletion UNFAVORABLE PROGNOSIS: 1. +TERT-mutant
59
Atypical HUS
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
Binemetinib
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
Complete Hyadiform Mole (CHM)
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
Adjuvant therapy for Low-Grade Gliomas s/p resection
RT followed by PCV (procarbazine, CCNU and vincristine)
63
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.
Angioimmunoblastic T-cell Lymphoma
64
1st-line treatment for Recurrent/Metastatic BOT SCC with PD-L1 CPS 1%
Pembrolizumab +cisplatin or carbo+5FU
65
Dexrazoxane
Used for primary prevention against anthracycline-induced myocardial toxicity
66
Morphological abnormality seen on peripheral blood smear in patients with Bernard Soulier syndrome
Giant Platelets and thrombocytopenia
67
RCC Clinical RIsk Stratification
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
Treatments for metastatic gastric GIST
1st-line: Imatinib 2nd line: Sunitinib 3rd line: Regorafenib
69
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?
Discontinue Epo and test for anti-Epo antibodies to r/o pure red aplasia 2/2 anti-Epo Abs
70
Neuroendocrine Tumor Pathology Grading
71
WHO Classification of Pancreatic Neuroendocrine Tumors (pNETs)
72
Management of Carcinoid Tumor of the Appendix
* 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
Systemic Treatment Options for Advanced NETs (pNETs vs. Carcinoid)
74
Both auto-SCT and allo-SCT recipients are at increased risk for which chronic diseases?
1. CVD 2. Renal Disease 3. Diabetes
75
Management of patient with metastatic prostate cancer with bone-only mets currently treated with Lupron+Bicalutamide p/w slowly rising PSA and remains asymptomatic?
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
GPII/III Inhibitors (abciximab, eptifibitide, tirofiban) mimic which congenital platelet disorder?

Hematology/Medical Oncology ABIM (2 decks)

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