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Oncology > Oncology I > Flashcards

Oncology I Flashcards

1
Q

benign tumors and malignant tumors both form due to initial mutations in normal cell populations. what is the difference in the processes which form benign tumors vs. malignant ones?

A

after the initial mutation which causes tumor formation, malignant tumors will go on to develop additional mutations, overexpressions and altered enzyme pathways, they will develop the capacity for invasion and metasasis and acquire features of malignancy, benign tumors will maintain well-controlled growth and benign histologic features

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2
Q

put the following stages of tumor development in order: metastatic melanoma, dysplastic nevus, benign nevus, radial growth phase, vertical growth phase

A
  1. benign nevus
  2. dysplastic nevus
  3. radial growth phase
  4. vertical growth phase
  5. metastatic melanoma
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3
Q

what is the most commonly mutated oncogene?

A

RAS

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4
Q

how are growth factor receptors involved in squamous cell lung cancer and breast cancer?

A

Growth factor receptors are overexpressed in squamous cell lung cancer (ERBB1) and breast cancer (HER2/NEU)

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5
Q

describe how each of the following features are affected in malignancy:

  1. ratio of nucleus to cytoplasm
  2. appearance of the nucleus (3 changes)
  3. mitotic rate
A
  1. increased Nucleus:cytoplasm ratio
  2. nucleus is pleomorphic, hyperchromatic, with prominent nucleoli
  3. increased mitotic rate
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6
Q

what are “keratin peals”?

A

keratin pears are seen in squamous cell carcinoma, whorl of tumor cells with keratin production in the center

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7
Q

squamous cells have tonofilaments. what are tonofilaments?

A

tonofilaments are intercellular bridges which are perpendicular between tumor cells

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8
Q

what are the cells of origin in sarcomas?

A

mesenchymal tissue

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9
Q

desmoplasia

A

fibrosis and connective tissue growth seen in carcinomas

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10
Q

do most breast carcinomas arise from acini (glands) or from duct epithelium?

A

duct epithelium

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11
Q

why does desmoplasia occur?

A

tissue attempts to “contain” the tumor with a fibroblastic response at primary sites and metastatic sites

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12
Q

what changes must take place in tumor cells in order for them to invade the basement membrane and ECM? Mutations in what gene are typically involved?

A

Mutation of E-cadherin allows for loosening of tumor cells to help them prepare to invade the Basement membrane (mutations in SNAIL and TWIST, which regulate E-cadherin are also seen)

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13
Q

Degradation of the BM and ECM requires the tumor cells to begin expressing what types of proteins?

A

tumor cells will begin secreting collagenases, urokinase plasminogen activator, gelatinase, etc.

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14
Q

how do to surface proteins change to allow tumor cells to interact with ECM proteins?

A

tumor cells will begin expressing integrins which have affinity for laminin and other ECM proteins

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15
Q

what genes are critical in the tumor’s ability to promote vasculogenesis?

A

the tumor expresses VEGF and HIF-1A (hypoxia inducible factor), as well as angiogenin and TGF-a,B

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16
Q

why are chemokine receptors important in metastasis?

A

chemokine receptors on tumor cells bind to certain target tissues, i.e. tumor cells expressing CCR7 will bind at target tissues such as lung which express CCL21

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17
Q

do carcinomas travel through arteries, veins or lymphatics? what about sarcomas? what are the target sites of each?

A

carcinomas: travel through lymphatics to regional lymph nodes
sarcomas: travel through veins to lungs and bone

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18
Q

metastases to the lung most commonly arise from what sites of origin?

A

colorectal, breast, kidney, HCC

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19
Q

what cancers typically metastasize to the liver?

A

colorectal, pancreas, GI, lung breast

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20
Q

what cancers commonly metastasize to the bone?

A

breast, prostate, renal

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21
Q

what cancers typically metastasize to the brain?

A

lung, breast, melanoma, renal

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22
Q

Virchow’s node

A

supraclavicular node, metastasis from gastric adenocarcinoma

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23
Q

Krukenberg tumor

A

bilateral metastatic malignancy to the ovaries from primary GI malignancy

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24
Q

what are sentinel lymph nodes?

A

the first node that receives lymphatic drainage when dye is injected into the tumor, helps determine where cancer has/could metastasize

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25
Q

which of the following might you not treat immediately upon diagnosis: ALL or CLL. why? which is more aggressive? which is more curable?

A

CLL, if asymptomatic, does not require treatment

ALL: more aggressive, always treat, more curable

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26
Q

what is the difference between a lymphoma and a leukemia

A

leukemia: begins in the bone marrow or blood, lymphocytes and myeloid cells affected
lymphoma: begins in bone marrow, WBC affected are lymphocytes only (Not myeloid cells)

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27
Q

what immune cells are involved in ALL?

A

B-cells and T-cells

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28
Q

is lymphadenopathy seen more in ALL or CLL?

A

seen more in CLL because CLL involves mature lymphocytes

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29
Q

is this smear ALL or CLL? how do you know? what are the relevant features?

A

this is CLL, small, round lymphocytes, clumpy chromatin, NO nucleoli, presence of platelets

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30
Q

is this ALL or CLL? how do you know? what are the relevant features?

A

this is ALL, BIG lymphocytes, lighter “open chromatin,” nucleoli, no granules

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31
Q

risk factors for ALL

A

radiation exposure, trisomy 21

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32
Q

typical patient presentation of ALL

A

cytopenia: bleeding, infection, fatigue, dizziness
fever
bone pain
(lymphadenopathy is RARE)

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33
Q

state whether each of the following more closely aligns with presentation of ALL or CLL: weight loss over 5 years, rash, mediastinal mass, lymphadenopathy, anemia, ecchymoses

A

anemia, ecchymoses, rash & mediastinal mass: ALL

chronic weight loss & lymphadenopathy: CLL

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34
Q

diagnostic test for ALL

A

bone marrow biopsy with flow cytometry

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35
Q

therapy for ALL: describe drugs for each stage: induction, CNS therapy, intensification, maintenance

A

induction: prednisone, vincristine, doxorubicin, asparaginase, cyclophosphamide, dexamethasone
CNS therapy & intensification: chemo
maintenance: prednisone, methotrexate, mercaptupurine

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36
Q

state which drug (dexamethasone/prednisone, vincristine, doxorubicin, cyclophosphamide, asparaginase, methotrexate) is associated w/each MoA: A) directly toxic to lymphocytes B) folate antagonist C) alkylating agent inducing bulky DNA lesions D) inhibits microtubule polymerization E) depletes asparagine F) inhibits topoisomerase II

A

A) directly toxic to lymphocytes: prednisone/dexamethasone
B) folate antagonist: methotrexate
C) alkylating agent inducing bulky DNA lesions: cyclophosphamide
D) inhibits microtubule polymerization: vincristine E) depletes asparagine: asparaginase
F) inhibits topoisomerase II: doxorubicin

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37
Q

doxirubicin inhibits toperisomerase. what is topoisomerase?

A

topoisomerase II is an enzyme which relieves supercoil to allow for transcription. so inhibition of topoisomerase prevents uncoiling so the cells can’t transcribe and replicate

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38
Q

signs of DIC (labs)

A 
increased PTT
increased aPTT 
increased D-dimer 
decreased platelets
decreased fibrinogen
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39
Q

clinical signs of DIC

A

petechiae, bruising, splinter hemorrhages, diaphoresis

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40
Q

what is tumor lysis syndrome? how does it present?

A

when tumor dies (from chemo or outgrowing blood supply, etc) cytokines and other cellular products are released. can lead to hypotension, AKI, labs: increased K+, → creatinine → uric acid, → phosphorous, → LDH, ↔ calcium

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41
Q

which of the following are associated with a better prognosis: t(9,22) Philadelphia chromosome, t(4,11), or hyperdiploidy

A

t(9,22) Philadelphia chromosome, t(4,11) are associated with poor prognosis
hyperdiploidy: better prognosis

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42
Q

what age group is most likely to have hyperdiploidy and therefore the best prognosis in ALL

A

children ages 1-10

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43
Q

which is more common: ALL or CLL

A

CLL is the most common form of leukemia

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44
Q

symptoms of CLL

A

chronic weight loss, lymphadenopathy, anemia (dizziness, fatigue), or asymptomatic

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45
Q

Describe stages 0-4 of CLL

A

Stage 0: monoclonal lymphocytosis
Stage 1: lymphadenopathy
Stage 2: hepatosplenomegaly
Stage 3: hemoglobin <100K

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46
Q

What is immune thrombocytopenic purpura?

A

An autoimmune disease characterized by IgG against platelet antigens. Antibody bound platelets are consumed in the spleen resulting in thrombocytopenia.

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47
Q

3 mechanisms of thrombocytopenia in CLL

A

splenic sequestration, ITP and marrow involvement

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48
Q

when do you treat CLL?

A

when it’s symptomatic (fever, night sweats, stage 3/4 CLL, rapid doubling time of WBC, systemic lymphadenopathy)

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49
Q

how does rituximab work?

A

monoclonal antibody to CD20

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50
Q

Idelalisib and Ibrutinib are newly approved therapies for CLL. in what patients are the most commonly prescribed? they are effective in CLL with what mutation?

A

used in relapse patients and 17p patients

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51
Q

are most tumors clonal or polyclonal?

A

most tumors are clonal. polyclonal tumors are rare.

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52
Q

can separate mutations act synergistically to produce a more malignant effect than each produces individually?

A

yup

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53
Q

what effect will c-MYc activation have on tumor cells?

A

c-myc activation will increase rate of division

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54
Q

are hMSH2 and hMLH1 genes typically activated or suppressed in cancer?

A

hMSH2 and hMLH1 are mismatch repair genes, commonly suppressed in cancer

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55
Q

what is a “proto-oncogene”? how is proto-oncogene activation different in the presence of a wildtype allele?

A

a gene which, when ACTIVATED, contributes to tumorgenesis, it is genetically dominant, in most cases, only one copy of the proto-oncogene need be activated, the presence of a wt copy will not reduce the phenotype

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56
Q

how are “dominant negative p53 mutations” an “Exception” to the rule?

A

usually p53 mutations act as tumor suppressor genes, where both copies need be deleted/defective to have loss of function, p53 is a homo tetramer, and a mutuation in just a single subunit will cause a dysfunctional protein

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57
Q

are most cancers heritable

A

no. 10% are heritable

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58
Q

describe how the age of onset, number of independent tumors and tumor frequencies changes depending on if the retinoblastoma was sporadic or inherited

A

age of onset: sporadic 6 years old, inherited 2 years old

number of independent tumors: sporadic = single tumor in one eye, heritable = multiple tumors with both eyes affected

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59
Q

are all carriers affected in sporadic and heritable retinoblastoma?

A

sporadic: both mutations acquired somatically, so if only one mutation, no effect
heritable: one alteration is germ line, all carriers will be affected with average of 10 tumors/person, both eyes affected

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60
Q

what cells are affected in Rb patients with abnormalities on chromosome 13? compare/contrast sporadic vs. heritable cases

A

sporadic cases: only tumor cells have chromosome 13 abnormalities
heritable cases: all cells have chromosome 13 abnormalities

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61
Q

do cases of sporadic Rb have mutations in the same gene as familial Rb?

A

yes

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62
Q

is there a risk of developing other (non-eye) tumors in Rb patients?

A

some carriers of germline Rb mutations will develop osteosarcomas, but this is very rare

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63
Q

explain how the phosphorylation state of Rb changes during the cell cycle. what phosphorylates Rb?

A

Rb is hypophosphorylated in G0 and early G1
Rb is hyperphosphorylated in S phase and G2 phase
CDK4/cyclin D is the complex which phosphorylates Rb

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64
Q

what is E2F? is it growth promoting or inhibiting?

A

E2F promotes S-phase genes, it is controlled by Rb (when Rb is unphosphorylated it binds to E2F and inhibits transcription of S phase products)

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65
Q

how could “occupancy of the Rb pocket” cause disease?

A

a virus (i.e. HPV) or other DNA virus protein can bind to the binding site on E2F, so that Rb can’t regulate it, this prevents Rb from being able to suppress transcription

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66
Q

what is p16? is p16 an oncogene or tumor suppressor?

A

p16 inhibits CDK4 (which deactivates Rb), it is a tumor suppressor

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67
Q

in retinoblastoma: is the Rb gene mutation dominant positive, dominant negative or recessive negative?

A

recessive negative (2 hits)

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68
Q

are p53 polypeptides more unstable when mutated or wildtype?

A

wildtype p53 polypeptides are more unstable

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69
Q

is MDM2 an oncoprotein or tumor suppressor? what effect does it have on p53?

A

oncoprotein, it inhibits p53

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70
Q

are p53 mutations common?

A

p53 mutations occur in most cancers

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71
Q

leukemia vs. lymphoma

A

leukemia: malignant lymphocytes in circulation, involvement of bone marrow
lymphoma: malignant transformation and proliferation in the lymph nodes

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72
Q

besides lymph nodes, what other parts of the body can be affected by lymphoma?Â

A

spleen can become enlarged, SVC syndrome (superior vena cava blockage), CSF involvement

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73
Q

presentation of someone with B cell lymphoma

A

lymphadenopathy, splenomegaly, anemia (fatigue), fever, weight loss, pruritis

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74
Q

what paraneoplastic syndromes can be seen in B cell lymphoma

A

nephrotic syndrome, hemolytic anemia, ITP

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75
Q

workup for someone with suspected B cell lymphoma: what imaging and labs would you order?

A

PET/CT to visualize lymphnodes

labs: LDH, Serum protein electrophoresis, CBC, ESR, lumbar puncture, test for viral infection

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76
Q

diagnosis of B cell lymphoma

A

excisional or core biopsy of hottest node, then workup: histologically, immunochemistry, cytogenetics, PCR, FISH

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77
Q

Stages of lymphoma: where is there involvement at each stage 1-4?

A
  • 1 malignant cells confined to a single node
  • 2 two or more lymph nodes are affected confined to one side of diaphragm
  • 3 lymph nodes above and below diaphragm
  • 4 multiple or disseminated foci of involvement
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78
Q

What criteria define one’s score (0-5) in IPI (international prognostic index) for B cell lymphoma?Â

A

Age (>60), serum LDH, ECOG status, Ann Arbor Stage, # of extra-nodal disease sites

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79
Q

What is the ECOG performance score 0-4? what does it attempt to gauge?

A

ECOG gauges how well a patient is feeling and how well they will potentially respond to treatment.
0 = fully active
1 = some symptoms
2 = Needs some assistance
3 = Needs complete assistance
4 = Near death
->ECOG3 - may not want to use chemotherapy because they may be too weak to handle it

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80
Q

what is the most common lymphoma?

A

diffuse large B-cell non-Hodgkin lymphoma

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81
Q

morphological characteristics of diffuse large B cell lymphoma

A

large lymphocytes 4-5x regular size

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82
Q

state which is a characteristic of Germinal Center B cell lymphoma or Non-Germinal center B cell lymphoma: 1)decreased suppression of NFKB 2) BCL6 dysregulation via silencing by p53

A

Germinal Center B cell Lymphoma: p53 silencing of BCL6

Non-GC B cell lymphoma: overactivation of NFKB

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83
Q 
R-CHOP is a common treatment regiment for DLBCL. what is the mechanism of action for each of the R-CHOP drugs?
R- rituximab
C -cyclophosphamide
H- hydroxyydanourubacin (doxorubicin)
O -oncovin (vincristine)
P - prednisone
A

R- rituximab: anti CD20
C -cyclophosphamide: alkylating agent
H- hydroxyydanourubacin (doxorubicin): topomerase II inhibitor
O -oncovin (vincristine): anti-microtubular
P - prednisone: direct toxicity to lymphocytes

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84
Q

why complication can sometimes arise with rituximab treatment since it is a human-mouse chimera? what additional complications can arise

A

allergic reaction, can be pre-treated with Tylenol/Benedryl, additional complications: tumor lysis syndrome, PML, reactivation of HBV, hypogammaglobulinemia

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85
Q

Is HIV-associated DLBCL Germinal Center Type or Non-Germinal Center/ABC Type?

A

HIV-associated DLBCL can be either germinal center type (usually when HIV well-controlled) or it can be non-germinal center/ABC Type (usually when newly diagnosed or poorly controlled)

86
Q

post-transplant DLBCL is associated with infection with what virus?

A

EBV virus

87
Q

describe a typical patient presentation in primary mediastinal large B cell lymphoma?

A

young female with SVC and a large mediastinal mass

88
Q

what is “double hit” lymphoma? what genes are involved? is slow growing or aggressive?

A

double hit lymphoma: translocations in MYC & BCL2 OR MCY & BCL6, aggressive, fast-growing

89
Q

what is double protein DLBCL? is it associated with a good prognosis or poor prognosis?

A

high protein expression of MYC & BCL2, associated with poor prognosis

90
Q

In immune-deficiency associated DLBCL, which immune cell population is depleted?

A

T-cells

91
Q

clinical feature of Burkitt’s lymphoma

A

rapid lymphadenopathy, EBV infection with splenomegaly

92
Q

molecular marker of Burkitt’s lymphoma? is it positive or negative for CD19 and CD20?

A

cMYC translocation, CD19+ and CD20+

93
Q

treatment of Burkitt’s lymphoma

A

CHEMO regimen (CODOX-M), cyclophosphamide, vincristine, doxorubicin, methotrexate

94
Q

The 3 varieties of Burkitt’s lymphoma are 1) HIV-associated 2) African Endemic 3)Sporadic.
A. which is the most common lymphoma in children?
B. Which type always presents with EBV infection?
C. which involves mandibular mass?

A

A. most common lymphoma in children is SPORADIC Burkitt’s lymphoma
B. African Endemic Burkitt’s Lymphoma ALWAYS presents with EBV
C. African Endemic involves a mandibular mass

95
Q

What are the 4 varieties of Mantle Cell lymphoma? why is their varying Ki67 expression significant?

A

4 varieties of mantle cell lymphoma: blastic, aggressive, indolent and leukemic
-blastic has most Ki67 expression and leukemic the lowest, reflects degree of cellular proliferation

96
Q

molecular markers of Mantle cell lymphoma

A

CyclinD1+, CD5+, CD23(-)

97
Q

treatment of mantle cell lymphoma

A

treat with chemo if it’s aggressive, watch and wait if it’s indolent
relapse: give bortexomib or ibrutinib/idelalisib

98
Q

is follicular lymphoma rapidly growing or slow growing?

A

follicular lymphoma is slow growing

99
Q

molecular marker of follicular lymphoma

A

BCL2 translocation, CD19+, CD20+, BCL6+

100
Q

treatment of follicular cell lymphoma

A

watch & wait or Rituximab CHOP (can do radiation if stage 1 detection)

101
Q

describe the features of Lymphoplasmacytic Lymphoma/Waldenstroms Macroglobulinemia

A

-hyperviscosity due to lots of IgM secretion, oozing blood

102
Q

cause of MALT lymphoma

A

antigen in a malt tissue i.e. H pylori

103
Q

molecular marker of MALT lymphoma

A

increased NFKB, CD19+, CD20+, CD5(-), CD23(-), CD10(-)

104
Q

treatment of MALT lymphoma

A

treat underlying cause if possible (i.e. Abx for H pylori), can also do chemo, surgery

105
Q

name the lymphoma and describe its features

A

lymphoplasmacytic lymphoma/waldenstrom’s macroglobulinemia: hyperviscosity, secretes IgM

106
Q

what can ibrutinib and idelalisib be used for?

A

used in mantle cell lymphoma relapse patients

107
Q

what exposures increase the risk of one developing AML?

A

ionizing radiation, alkylating agents, topoisomerase inhibitors (chemotherapy), organic solvents

108
Q

what inherited disorders predispose one to AML?

A

Down’s syndrome, Fanconi’s syndrome (DNA repair defect), Li-Fraumeri syndrome (p53 mutation), hemetologic disorders

109
Q

clinical presentation of AML

A

fatigue, bruising, dyspnea, fever, bone pain, pallor, hemorrhage, ecchymoses, infection, hepatosplenomegaly skin or gum infiltration

110
Q

AML M2: what is the underlying genetic cause? is this associated with good prognosis or poor prognosis? what population is affected?

A

translocation (8:21), most common AML, seen in younger patients, myeloid maturation preserved, good prognosis, genetic cause: fusion of RUNX1 and RUNX1T1

111
Q

what drugs are used in induction therapy for AML?

A

cytarabine and anthracycline

112
Q

what is the MoA of cytarabine?

A

inhibits DNA and RNA polymerase

113
Q

side effects of cytarabine in the treatment of AML

A

GI (inflammation, ulceration, diarrhea), bone marrow suppression (leukopenia, thrombocytopenia anemia)

114
Q

mechanism of action of daunorubicin/idarubacin

A

intercalating agent, topoisomerase II inhibitor

-metabolized in liver so dose appropriately in hepatic dysfunction

115
Q

side effects of daunorubicin/idarubicin

A

bone marrow suppression, alopecia, GI side effects, cardiac toxicity

116
Q

why are people are higher risk of infection during aplasia?

A

in aplasia, the chemo has wiped out blast cells and hematopoietic elements, the patient isn’t making any blood cells after chemo, and this put them at risk of infection

117
Q

what age range is most affected by AML?

A

65+

118
Q

If intensive induction therapy for AML is too intense for the patient (i.e. patient is over 80 years old, has high ECOG score or a complex karyotype), what are the alternate treatment options?

A

older patients and more fragile patients can receive hypomethylating agent (decitabine, azacitidine)

119
Q

how does acute promyelocytic leukemia present?Â

A

bleeding diathesis, hypocoagulability and increased bleeding

120
Q

what is the target binding site of ATRA (all trans retinoic acid)? what is the role of ATRA in treatment of acute promyelocytic leukemia?

A

ATRA (all trans retinoic acid) binds to RAR (retinoic acid receptor) which relieves the previously bound co-receptors and allows transcription so that immature leukemic myelocytes can become mature granulocytes, when RAR binds co-repressors, they remain leukemic WBCs

121
Q

which is the more effective treatment in acute promyelocytic leukemia: retinoic acid (ATRA) or chemo?

A

ATRA is more effective in preventing relapse in PML patients

122
Q

what is the pathogenesis of APL differentiation syndrome?

A

inflammatory response provoked by leukemic cells in the process of differentiation, occurs after induction of therapy, after co-repressors are relieved and free to transcribe, there is a spike in number of differentiated WBCs causing the leukocytosis

123
Q

signs of APL differentiation syndrome

A

fever, dyspnea, weight gain, pulmonary infiltrates, pulmonary and pericardial effusions

124
Q

treatment of APL differentiation syndrome

A

dexamethasone intravenously

125
Q

myelodysplastic syndrome

A

increased proliferation, increased apoptosis
pre-leukemia”: heterogeneous clonal neoplastic bone marrow disorder which causes ineffective hematopoesis, increased risk of progression to AML “

126
Q

5q syndrome is the deletion of chromosome 5q. what are the clinical manifestations of this syndrome? is it more common in men or women? will a person with 5q syndrome progress to AML?

A

5q syndrome is deletion of 5q resulting in anemia and thrombocytopenia, seen mainly in WOMEN: low rate of progression to AML

127
Q

lenalidomide can be used as a treatment for 5q syndrome. lenalidomide is derived from what drug?

A

thalidomide

128
Q

Treatment options (A) Low Lenalidomide, or High Azacitidine or Decitabine (B) Lenalidomide (C)Supportive care (D) Allogenic Hematopoetic Cell Transplant

A

Young HLA matched donor = HCT
No HLA match, stable = supportive care
No HLA match, young or progressive disease = Low Lenalidomide, or High Azacitidine or Decitabine
5q syndrome = lenalidomide

129
Q

what is the only curative treatment for myelodysplastic syndrome?

A

allogenic HCT

130
Q

peripheral T cell lymphoma basics: A) is it common or rare? B) where in the world is it most common? C) what is the standard treatment regimen? D) Are they indolent or aggressive? E) Is the prognosis typically better or worse than B-Cell lymphoma?

A 
Peripheral T Cell Lymphoma
A) is rare (5% of NH Lymphoma)
B) is most common in Asia
C) no standard treatment regimen
D) range from indolent to aggressive
E) worse prognosis than B cell lymphoma
131
Q

do ALK+ ALCL have a better prognosis or worse prognosis than other subtypes of Peripheral T cell lymphoma

A

ALK+ has better prognosis (5 year survival 65-90% compared to 26%)

132
Q

what drug can be used to treat ALK+ ALCL?

A

brentuximab (anti-CD30)

133
Q

what feature of ALK+ ALCL makes it targetable for treatment?

A

ALK+ peripheral T cell lymphoma is CD30+ so it can be treated with brentuximab

134
Q

what histological characteristic is typical for anaplastic large t cell lymphoma?

A

horseshoe nuclei

135
Q

in general, patients with T-cell lymphoma will present with what clinical sign?

A

rash (some others will have lymphadenopathy, splenomegaly)

136
Q

is CHOP therapy effective in T cell lymphoma?

A

no. CHOP is not effective in T cell lymphoma

137
Q

what specific cytogenetic markers help diagnose T cell lymphoma?

A

there are no specific cell markers for T cell lymphoma, T cells in lymphoma do have loss of surface markers and do not rearrange like normal T cells

138
Q

what abnormality drives Adult T cell leukemia/lymphoma?

A

HTLV-1 virus

139
Q

how is HTLV-1 virus transmitted?

A

breastmilk, needles sexual intercouse

140
Q

what is the classic histological feature of Adult T Cell leukemia/lymphoma?

A

flower cell morphology

141
Q

how long after infection with HTLV-1 will a patient develop Adult T Cell leukemia/lymphoma?

A

latency period of 10-30 years

142
Q

From 1992-2007, there have been 89 cases of HTLV-1 ATLL in NYC. What countries were the patients predominantly from?

A

26% from Jamaica
18% from DR
7% African American

143
Q

presentation of HTLV1-ATLL

A

lymphadenopathy, rash, increased calcium levels, CNS involvement

144
Q

Therapy for HTLV1 ATLL

A
  1. chemotherapy
  2. anti-viral therapy (zidovudine + IFN)
  3. transplant
  4. anti-CD25 and anti-CCR4 antibodies, bortexomib, lenalidomide
145
Q

does the addition of etoposide during treatment improve or worsen the course of HTLV1 ATLL?

A

adding etoposide improves overall survival in HTLV1 ATLL

146
Q

HDAC inhibitors are approved for relapse settings. how do they work?

A

histone de-acetylase inhibitors keep tumor suppressor chromatin open for transcription

147
Q

what cell surface protein is found on all Hodgkins lymphomas? what drug takes advantage of this featured?

A

CD30, targeted by brentuximab (anti CD30 monoclonal antibody), used in relapse settings

148
Q

What distinguishes Hodgkins lymphoma from non-hodgkins?

A

Hodgkins lymphoma: contains Reed Sternberg Cells, cell of origin is most likely a B-cell post germinal-center

149
Q

EBV infection is typically seen in Hodgkin’s lymphoma. what pathway is upregulated as a result?

A

the NFKB pathway is unregulated as a result of EBV infection in hodgkin’s lymphoma

150
Q

The 4 subtypes of Hodgkins lymphoma are:
1. nodular sclerosis
2. mixed cellularity
3. lymphocyte rich
4. lymphocyte depleted
Which is most the most common type? which is associated mainly with HIV patients?

A

nodular sclerosis is the most common Hodgkins subtype

mixed cellularity is HIV-associated

151
Q

classic Hodgkins lymphoma presentation (age, symptom)

A

young adult, presents with large mediastinal mass, pruritis

152
Q

what is the effect of alcohol on patient’s with hodgkin’s lymphoma?

A

burning of lymph nodes upon alcohol consumption

153
Q

Treatment regimen for hodgkin’s lymphoma and name the MoA

A 
ABVD:
Adriamycin (anthracycline)
Bleomycin (anti-neoplastic)
Vinblastine (anti-microtubule)
Dacarbazine (alkylating agent)
154
Q

which drug in the ABVD therapy is associated with cardiotoxicity? which is associated with neuropathy? which with pulmonary fibrosis?

A

adriamycin: cardiotoxicity
vincristine: anti-microtubule
bleomycin: pulmonary fibrosis

155
Q

If a patient with HL relapses post-chemo, what therapy is most effective?

A

transplant can be curative in relapsed Hodgkin’s patients

156
Q

lymphocyte-predominant Hodgkin Lymphoma presents in young males with lymphadenopathy in what region? how is this disease treated? is it associated with EBV?

A

lymphocyte predominant Hodgkin lymphoma is rare, and presents in young males as cervical lymphadenopathy. it is treated with rituximab, NOT associated with EBV

157
Q

if this graph depicts the course of Multiple myeloma, what is being measured on the Y axis?

A

M-protein levels

158
Q

describe the pathophysiology of multiple myeloma. what are M proteins?

A

in multiple myeloma, malignant plasma cells secrete M proteins. M proteins pathogenic antibodies which can be IgA, IgG or free light chains (kappa or lambda). Light chain deposition can cause renal failure. Bone resorption also occurs, with lytic lesions, and normal immune function is suppressed

159
Q

is multiple myeloma an acute disease or a chronic disease?

A

chronic disease

160
Q

what racial group is most affected by multiple myeloma? what age group most affected?

A

African Americans, low risk in asians,

Multiple myeloma seen in older adults (median age 66)

161
Q

clinical presentation of multiple myeloma

A 
CRAB
Calcium increase
Renal damage
Anemia
Bone lytic lesions
(also, weight loss and fatigue)
162
Q

diagnosis of multiple myeloma: 3 components

A
  1. bone marrow histology
  2. monoclonal immunoglobulins in blood, urine
  3. imaging: X Ray (not MRI), we will see bone breakdown
163
Q

explain how this serum protein electrophoresis would look different in a multiple myeloma patient

A

in a multiple myeloma patient, there would be a thick band at the site representing the weight of the M protein produced (i.e. if it was a IgG secreting MM, there would be a large band on the anion side of the gel)

164
Q

what is MGUS? what is the difference between MGUS and smoldering myeloma or active myeloma?

A

MGUS: monoclonal gammopathy of undetermined significane
in MGUS, there is a hyperproduction of M protein, but the production levels are not as high as in myeloma and there is no end organ damage

165
Q

do most patients with MGUS progress to multiple myeloma?

A

No. there is a 30% risk of progression to multiple myeloma over 30 year period

166
Q

how does the size of the M-protein produced in multiple myeloma affect prognosis?

A

the bigger the protein, the higher the risk of progression to myeloma or related malignancy

167
Q

presentation and treatment of smoldering myeloma

A

asymptomatic, wait to treat until progresses to symptomatic disease

168
Q

Indications for treatment of Multiple Myeloma

A

M-CRAB
M: extraMedullary plasmacytoma
C: calcium in blood elevated
R: renal insufficiency, with elevated creatinine
A: anemia
B: Bone contains lytic lesions, osteopenia

169
Q

the stage of disease for Multiple myeloma is based on what major factor?

A

stage of multiple myeloma depends on B2 microglobulin levels ( 5.5 in stage 3), also depends on albumin level

170
Q

if a patient is too old to undergo transplant, what are the treatment options for MM?

A

treatment options are MP, MPR and VMP (chemo drugs)

171
Q

what would make a patient ineligible for autologous transplant?

A

age 77+, increased direct bilirubin, creatinine, ECOG score

172
Q

how do bisphosphonates act in treatment of multiple myeloma

A

bisphosphonates inhibit osteoclast activity to prevent lytic lesions in bone and prevent osteopenia

173
Q

complication of bisphosphonate therapy

A

osteonecrosis of the jaw

174
Q

therapy for multiple myeloma if chemo and transplant do not work. what drug can be used?

A

bortezomib (proteasome inhibitor) can be used in multiple myeloma

175
Q

explain the basic pathophysiology of CML. what cells are over-proliferating?

A

BCR-ABL fusion gene causes activation of kinase pathways, leads to overgrowth of granulocyte and megakaryocyte precursors in the bone marrow

176
Q

epidemiology of CML

A

45-55 years old, most present asymptomatically, 85% diagnosed in chronic stage of disease via routine labs

177
Q

symptoms and signs of CML

A

many are asymptomatic, symptoms include: fatigue, abdominal fullness (due to hepatosplenomegaly), night sweats, weight loss, fever, anemia, ecchymoses, thrombocytosis, leukocytosis

178
Q

lab findings in CML

A

increased myeloid cells (mature & immature), basophilia, anemia, thrombocytosis

179
Q

how can you evaluate disease burden in CML?

A

check for the number of BCR-ABL transcripts in the bone marrow

180
Q

describe the 3 phases of CML and their durations

A
  1. chronic phase (4-6 years)
  2. Advanced: accelerated phase (usually around 1 year)
  3. Advanced: blast crisis (survival 3-6 months)
181
Q

types of therapies for CML (3)

A
  1. small molecule inhibitor: imatinib, nilotinib
  2. interferon
  3. stem cell transplant
182
Q

imatinib mechanism of action, where is it metabolized? what are the side effects?

A

binds to the ATP binding site on BCR-ABL fusion to block ATP binding, therefore prevents downstream signaling
metabolism: liver
Side effects: generally well-tolerated with some GI side effects, leukopenia, anemia, thrombocytopenia

183
Q

indications for imatinib treatment

A
  1. CML
  2. Ph+ ALL
  3. GIST
184
Q

how do imatinib, nilotinub and desatinib vary?

A

nilotinib is more specific in its target molecules compared to imatinib, desatinib hits a wider range of targets but has increased side effects

185
Q

describe the general pathogenesis of polycythemia vera

A

overproliferation of red blood cells due to JAK2 mutation, erythropoetin-independent production by myeloid progenitors, complications include abnormal blood flow leading to bleeding and thrombosis-related complications

186
Q

who gets polycythemia vera?

A

older adults 40+, slight male predominance

187
Q

symptoms of polycythemia vera

A

pruritis, plethora (ruddy complexion), abdominal fullness due to splenomegaly

188
Q

genetic cause of polycythemia vera

A

JAK2 mutation

189
Q

bone marrow features in polycythemia vera

A

hypercellularity, low iron stores in RBC, large, clustered megakaryocytes

190
Q

are the over-produced red blood cells mature or immature in polycythemia vera?

A

RBC are at full maturity in polycythemia vera

191
Q

diagnosis of polycythemia vera requires either 2 major criteria be met or 1 major criteria and 2 minor criteria. what are the 2 major criteria? what are the 3 minor criteria?

A 
Major criteria for polycythemia vera
1. increased Hgb
2. JAK2 mutation 
Minor criteria
1. hypercellular marrow
2.low Erythropoietin
3. endogenous erythroid colony formation in vitro
192
Q

when polycythemia vera is suspected, what else is on the DDX?

A

chronic hypoxia (due to high altitude, cigarettes, pulmonary disease, sleep apnea), EPO abuse, familial erythrocytosis (mutation in the Epo receptor)

193
Q

complications of polycythemia vera

A

bleeding, thrombosis, transformation to AML, myelofibrosis (seen in older patients)

194
Q

treatment of polycythemia vera

A
  1. standard: phlebotomy to decrease HgB to normal level & low dose aspirin
  2. if patient has thrombotic event OR severe splenomegaly OR cannot tolerate phlebotomy, give cytoreductive agent (interferon alpha or hydroxyurea for older patients)
195
Q

general pathogenesis of essential thrombocytosis

A

JAK2 mutation or MPL (TPO receptor) is overactivated causing excess Megakaryocyte progenitor proliferation, leads to increased risk of thrombosis

196
Q

who gets essential thrombocytosis

A

older adults (60+)

197
Q

symptoms of essential thrombocytosis

A

pruritis, erythromelalgia (pain in hands and feet), abdominal fullness (splenomegaly)

198
Q

if JAK2 mutations cause both polycythemia vera and essential thrombocytosis, what determines if the patient develops one disease versus another?

A

the same gene is mutated in both diseases but the subsequent pathogenesis mechanisms are not understood, there is some overlap in disease

199
Q

diagnosis of essential thrombocytosis (4 criteria)

A
  1. increased platelet count (>450K)
  2. bone marrow shows megakaryocyte proliferation
  3. no evidence of polycythemia vera or CML, etc.
  4. JAK2 or MPL mutation OR no reactive thrombocytosis
200
Q

elevated platelet count can be seen in what other conditions

A

iron deficiency, inflammation, surgery, splenectomy, metastatic cancer, CML, polycythemia vera

201
Q

complications of essential thrombocytosis

A

thrombosis, bleeding, myelofibrosis, AML

202
Q

management of essential thrombocytosis

A
  1. manage CV risk factors aggressively to decrease thrombotic risk
  2. low dose aspirin
  3. high risk only: add hydroxyurea or interferon
203
Q

general pathogenesis of primary myelofibrosis

A

JAK or MPL mutation causes hypercellularity in bone marrow (leukocytosis and thrombocytosis), since there are lots of megakaryocytes secreting PDGF, this activates reactive fibroblasts and eventually the marrow is replaced with fibrosis, this causes burden of hematopoiesis to shift to the spleen and liver

204
Q

who gets primary myelofibrosis?

A

older adults

205
Q

signs & symptoms of primary myelofibrosis

A

SPLENOMEGALY, anemia (fatigue, pallor, dyspnea), bleeding, bruising, infection, cachexia (abdominal fullness due to splenomegaly)

206
Q

why is this man’s abdomen distended in primary myelofibrosis?

A

hematopoesis is taking place in the spleen and liver since bone marrow is fibrosed

207
Q

what do blood smears look like in primary myelofibrosis? what is the histological hallmark?

A

tear drop shaped RBCs seen in primary myelofibrosis, the blood will also look like marrow since there will be leukoerythroblastic features

208
Q

bone marrow features in primary myelofibrosis

A

megakaryocyte hyperplasia, pleomorphic megakaryocytes, granulocytes intact, fibrosis, increased osteoblasts and osteoclasts

209
Q

diagnosis of primary myelofibrosis: what 3 major criteria are required?

A
  1. hypercellular marrow with weird megakaryocytes and fibrosis
  2. no polycythemia vera, CML, ET
  3. JAK2 mutation
    minor criteria (2 required): leukoerythroblastosis, elevated LDH, anemia, splenomegaly
210
Q

calreticulin mutations are seen in most cases of essential thrombocytosis and primary myelofibrosis. what is calreticulin?

A

calreticulin regulates gene transcription, it is an auto-antibody target in SLE and Sjogrens, major calcium storage protein in ER

211
Q

management of primary myelofibrosis

A

EPO transfusions, hydroxyurea (to decrease WBC count), splenectomy, SCT, JAK inhibitors

Oncology (5 decks)

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