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Oncology > Onc Exam > Flashcards

Onc Exam Flashcards

1
Q

What is proliferation?

A

How quickly cells divide

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2
Q

What is maturation (re malignancy)?

A

How much the malignant cells resemble normal cells
•Malignant cells blocked in an immature stage always behave aggressively.
•Malignant cells able to mature sometimes behave aggressively.

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3
Q

Which cells are derived from lymphoid progenitor?

A

b lymphocyte
t lymphocyte
natural killer cells
plasma cells

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4
Q

Which cells are derived from myeloid progenitor?

A 
RBC
platelets
monocytes
PMNs
eosinophils
basophils
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5
Q

Which hematologic malignancies display normal maturation and increased proliferation?

A 
CLL
CML
myeloproliferative neoplasms (MPN)
multiple myeloma
indolent lymphoma
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6
Q

Which hematologic malignancies display immature maturation and normal proliferation?

A

Myelodysplastic syndrome (MDS)

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7
Q

Which hematologic malignancies display immature maturation and increased proliferation?

A

ALL
AML
high-grade lymphomas (Burkitts, DLBCL)

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8
Q

What are the most common malignancies?

A

lung

breast

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9
Q

Which is the number 1 cause of death due to malignancy?

A

lung cancer

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10
Q

In which malignancies has the death rate not particularly changed in the last 50+ years?

A

pancreas

liver

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11
Q

What is survival time?

A

time from initial diagnosis to death

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12
Q

What is disease-free survival?

A

Time from remission to disease relapse

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13
Q

What is the mortality rate?

A

Number of deaths within a given population in a given time period. Mortality rate is typically expressed as number of deaths per 1,000 or 100,000 individuals per year.

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14
Q

What does the APC deletion result in?

A

Inherited (germ line) mutation

Results in familial adenomatous polyposis (colon cancer)

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15
Q

What does the TP53 mutation result in?

A

Inherited (germ line) mutation

Results in Li-Fraumeni (breast cancer, leukemia, sarcoma, etc)

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16
Q

Which malignancies are increased by tobacco?

A

Lung
head and neck
bladder
pancreas

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17
Q

How does staging work?

A 
TNM Staging
T: Tumor Size/Involvement
N: Nodal Status
M: Distant Metastatic Disease
These values correlate with a stage: 0-IV
Typically 0: in situ cancer
IV: metastatic disease
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18
Q

What is a prognostic factor?

A

factor that provides info on likely outcome in an untreated patient

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19
Q

What is a predictive factor?

A

factor that provides info on the likely benefit of treatment

examples: estrogen receptor status, EGFR status

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20
Q

How do cancers metastasize?

A

Each cancer can spread through one or more of these routes:
•Direct extension into adjacent tissues: lung, gynecologic cancer
•Lymphatic: breast, lung, colorectal, prostate cancers
•Hematogenous: sarcomas, kidney cancer

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21
Q

Which cancers typically met to the brain?

A

lung, breast, melanoma, renal cell ca

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22
Q

Which cancers typically met to the lung?

A

breast, colorectal, renal cell ca, HCC

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23
Q

Which cancers typically met to the liver?

A

colorectal, pancreatic, breast, lung, other GI

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24
Q

Which cancers typically met to the bone?

A

breast, lung, kidney, prostate

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25
Q

When might you see hypercalcemia in cancer?

A

PTHrP in non small cell lung cancer

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26
Q

When might you see cushing’s syndrome in cancer?

A

ACTH in small cell lung cancer

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27
Q

When might you see SIADH in cancer?

A

small cell lung cancer

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28
Q

What is the predominant site of involvement in leukemia?

A

blood and bone marrow

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29
Q

What is the predominant site of involvement in lymphoma?

A

lymph nodes

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30
Q

What are risk factors for ALL (acute lymphoblastic leukemia)?

A

radiation exposure

trisomy 21

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31
Q

How do patients with ALL (acute lymphoblastic leukemia) present?

A

Acute complications of cytopenias (bleeding, infection, fatigue, dyspnea, dizziness)
Fever
Bone pain (bone marrow expands as leukemia grows)
Lymphadenopathy is rare

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32
Q

What is seen on smears in ALL?

A 
Very big WBCs: Large redundant lymphocytes
Open chromatin
Prominent nucleoli
Light purple cytoplasm
No granules
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33
Q

What might be seen on physical exam in ALL?

A 
Signs of anemia
Ecchymosis, petechiae
Lymphadenopathy
Splenomegaly
Rash
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34
Q

How is ALL diagnosed?

A

Bone marrow tests:

  1. Core biopsy
  2. Flow cytometry
  3. Aspirate slides
  4. Cytogenetics
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35
Q

What is flow cytometry?

A

Flow Cytometry: identification of cell surface proteins with fluorescence-labeled antibodies.
CD19 is particularly looked for

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36
Q

How does T-cell ALL typically present?

A

mediastinal mass

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37
Q

How do steroids work in ALL?

A

directly toxic to lymphocytes (induce apoptosis)

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38
Q

How does vincristine work?

A

Inhibits microtubule polymerization

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39
Q

How does doxorubicin work?

A

Inhibits topoisomerase II

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40
Q

How does cyclophosphamide work?

A

alkylating agent - induces bulky DNA lesions

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41
Q

How does asparaginase work?

A

Depletes asparagine

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42
Q

How does methotrexate work?

A

Folate antagonist

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43
Q

What are major complications of ALL?

A
  1. DIC (PT up, aPTT up, fibrinogen down, d-dimer up, platelets down)
    Treat with cryoprecipitate
  2. Tumor lysis syndrome
    Treat with allopurinol and rasburicase
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44
Q

What are adverse prognostic factors for ALL?

A

Being an adult, particularly 60+ years old
Adverse cytogenetics
B-cell phenotype
Presence of minimal residual disease

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45
Q

What are prognostic cytogenetics in ALL?

A

t(9;22): Philadelphia chromosome (BAD)
t(4;11): bad
hyperdiploidy: good

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46
Q

When should patients with ALL receive allogeneic transplant?

A

Widespread acceptance in young patients with adverse cytogenetics in first remission.
Standard treatment for relapsed ALL in young patients.
Important factors in other scenarios:
- Patient age
- Presence of HLA-matched sibling

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47
Q

What is minimal residual disease?

A

Molecular minimal residual disease (MRD) is the percentage of residual leukemia cells in the bone marrow, and determines risk of relapse.

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48
Q

What is CLL?

A

Most common form of leukemia
Only form of leukemia without increased incidence in atomic bomb survivors.
A disease of the elderly.

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49
Q

What is seen on smear in CLL?

A 
•Small redundant lymphocytes (nucleus is about the size of an erythrocyte)
•Clumped chromatin
•Absent nucleoli
•Light purple cytoplasm
Much smaller than ALL lymphoblasts
Sometimes see smudge cells
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50
Q

How do CLL patients typically present?

A

Asymptomatic lymphocytosis
Lymphadenopathy is common
Complications of cytopenias (recurrent sino-pulmonary infections, gradual fatigue, dyspnea, dizziness)
Chronic weight loss

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51
Q

How is CLL staged?

A 
Stage 0: Monoclonal lymphocytosis 6,000+/μL
Stage 1: Lymphadenopathy
Stage 2: Hepatosplenomegaly
Stage 3: Hemoglobin less than 11 g/dL
Stage 4: Platelets less than 100,000
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52
Q

How can CLL disrupt the immune system?

A 
Immune Thrombocytopenic Purpura (ITP)
Autoimmune Hemolytic Anemia (AIHA)
Hypogammaglobulinemia
Pure red cell aplasia (PRCA)
Other auto-immune diseases
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53
Q

When should CLL be treated?

A 
Stage 3/4 CLL
Rapid Lymphocyte Doubling Time
Symptomatic Lymphadenopathy
Fevers, Night Sweats, Weight Loss
Transformation to High-grade Lymphoma
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54
Q

What is the only cure for CLL?

A

Allogeneic transplant is the only cure for CLL but carries a high mortality rate (treatment may be worse than disease in some cases).

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55
Q

How does AML typically present?

A 
Symptoms
•Fatigue
•Bruising/bleeding
•Dyspnea
•Fever
•Bone pain
Signs
•Pallor
•Hemorrhage
•Ecchymoses, petechiae
•Infection
•Hepatosplenomegaly
•Skin or gum infiltration
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56
Q

How do B cell lymphomas typically present?

A

Expansion of lymph nodes
- Enlargement of lymph nodes, spleen
- Superior Vena Cava (SVC) syndrome (compression of VC, leading to swelling of face and lower extremities)
- CSF involvement
Cytopenias (infections, anemia, bleeding, petechiae)

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57
Q

What are the “B symptoms” of lymphoma?

A

fever
weight loss
pruritus

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58
Q

How is lymphoma diagnosed?

A 
Excisional or Core Biopsy of hottest node
–Histologic examination
–Immunohistochemistry phenotype
–Cytogenetics
–Molecular tests (FISH, PCR)
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59
Q

How does staging work in lymphoma?

A

It’s kind of complicated, but ultimately it does not have a lot of prognostic value

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60
Q

What is diffuse large b-cell lymphoma?

A 
Most common Lymphoma:
Can arise anywhere in the body
•Morphology
–Large cell size (4-5x a normal lymphocyte)
–Diffuse growth pattern
•Immunohistochemistry
–(+)CD19 CD20
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61
Q

What are the 2 predominant drivers of diffuse large b-cell lymphoma?

A
  1. germinal center (GC) type: mutations lead to a repressed transcriptional state
  2. activated b-cell (ABC) type: mutations in the b cell receptor pathway lead to unchecked activation of NFkB
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62
Q

What is the molecular pathogenesis of GC-derived diffuse large b-cell lymphoma?

A
  1. enter the lymph node as naive B cell
  2. upregulated in germinal center, allowing for somatic hypermutation
  3. In normal B cell, factors are then downregulated; instead, EZH2 and Bcl6/2 are mutated and upregulated
  4. GC-derived lymphoid neoplasm
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63
Q

How is diffuse large b-cell lymphoma typically treated?

A 
RCHOP
- Rituximab
- Cytoxan
- Doxorubicin
- Vincristine
- Prednisone
\+ growth factor support
\+ antibiotic prophylaxis in the elderly
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64
Q

What is rituximab?

A
  • Monoclonal Antibody to CD20
  • Human-Mouse Chimera
  • Allergic reactions can occur (especially with first dose): pretreat with Tylenol/Benadryl
    –Tumor Lysis when Malignant Lymphocytes 25,000+
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65
Q

What are the special subtypes of diffuse large b-cell lymphoma?

A
  1. Immuno-deficiency associated (HIV, post-allo transplant, EBV)
  2. Primary effusion lymphoma (HIV, elderly, KSHV/HHV-8)
  3. Double hit lymphoma (typically seen in the elderly; poor prognosis)
  4. Double protein lymphoma: poor prognosis
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66
Q

What is primary mediastinal large b-cell lymphoma?

A 
•Probably more like Hodgkin's than DLBCL
–CD30+, CD20+, nuckeal c-rel, TRAF-1
•Female Young Adults
•Large mediastinal mass
–SVC syndrome, thrombosis common
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67
Q

What are the 3 variants of Burkitt’s?

A
  1. HIV associated
  2. African endemic (mostly children, mass involving mandible, abdominal viscera, kidneys, ovaries, adrenals)
  3. Sporadic (most common lymphoma in children; associated with t(8;14)
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68
Q

What is Burkitt’s Lymphoma?

A

Rapid enlargement of lymphadenopathy (DAYS)
All subtypes driven by c-MYC translocation
Often associated with EBV
all have Starry Sky appearance on histopath
Aggressive, but respond well to chemo

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69
Q

How does Burkitt’s lymphoma appear on histopath?

A

“Starry Sky”
–Effaced by diffuse infiltrate
–Intermediate size round or oval nuclei
–High mitotic index and numerous apoptotic cells
–Nuclear remnants are phagocytosed by benign macrophages

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70
Q

What is Follicular Lymphoma?

A 
Clinical Picture:
•Painless, generalized lymphadenopathy
•Incurable, median survival 7-9 years
•Indolent waxing and waning course
•Bone marrow involvement in 85%
•Histologic transformation in 30-50%
•Immunophenotype:
–(+) Bcl2
–(+) CD19, CD20, CD10, Bcl6
–(-) CD5
Bcl2
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71
Q

How is Follicular Lymphoma treated?

A
  • Watch and wait
  • Rituxan-based therapy
  • CHOP or CVP
  • Bendamustine
  • Purine analogue/Alkylating Agent hybrid
  • Cytopenias
  • XRT for stage I disease
72
Q

What does Follicular Lymphoma look like on histopath?

A

–nodular pattern
–small cells with irregular or cleaved nuclear contours
–Grade depending on # of large cells: 1-2, 3a, 3b

73
Q

What are the 4 variants of mantle cell lymphoma?

A

Blastic
Aggressive
Indolent
Leukemic (indolent, behaves like CLL)

74
Q

How is mantle cell lymphoma treated?

A 
•Treatment guided by variant
–Aggressive chemotherapy and transplant
–Bendamustine
–Bortezomib
–Ibrutinib and Idelalisib in relapsed setting
–Watch and Wait when indolent
75
Q

What is AML M2 and who gets it?

A 
AML
•Younger patients
•Extramedullary disease
•Favorable prognosis
•Fusion of RUNX1 and RUNX1T1 genes
76
Q

What is cytarabine?

A

Mechanism of action
•Converted to its active form, aracytidine triphosphate (ara-CTP)
•Incorporated into DNA, causing strand termination
•Inhibits DNA and RNA polymerase.
Metabolism
•Metabolized in the liver to inactive uracil arabinoside
•Excreted in the urine
•Often administered by continuous intravenous infusion because of its short half-life

77
Q

What are the side effects of cytarabine?

A
  • Gastrointestinal: inflammation of mucous membranes, sometimes with ulceration and diarrhea
  • Bone marrow suppression: leukopenia, thrombocytopenia and anemia
78
Q

What is daunorubicin?

A

Mechanism of action
•Intercalation into DNA, thereby impairing transcription
•Topoisomerase II inhibition
•Generation of free oxygen radicals
Metabolism
•Metabolized in the liver
•Dose must be adjusted when patients have significant hepatic dysfunction.

79
Q

What are the side effects of daunorubicin?

A
  • Gastrointestinal side-effects: nausea and vomiting, inflammation of the mucous membranes, diarrhea
  • Bone marrow suppression
  • Alopecia
  • Cardiac toxicity
80
Q

What are the adverse effect risk factors for survival in AML?

A
  • Age 80+ years
  • ECOG 2+ (poor functional status)
  • Complex karyotype
  • Creatinine 1.3+ mg/dl
81
Q

What is APL?

A

Acute Promyelocytic Leukemia (APL)
•10% of AML
•Presents with bleeding diathesis
•Characterized by t(15;17), resulting in PML/RAR-alpha fusion transcript
•Responds to retinoic acid and arsenic trioxide

82
Q

What is APL Differentiation Syndrome?

A

•Occurs in 30% of patients during induction
•Often associated with leukocytosis
•Requires prompt intervention
•Begin dexamethasone 10 mg intravenously twice daily
̶Continue for at least 3 days
•Can be fatal if treatment is delayed
Early symptoms include fever, dyspnea, and weight gain; late symptoms are pulmonary infiltrates and pleural and pericardial effusions

83
Q

What is MDS?

A

Myelodysplastic Syndrome (MDS)
•Heterogeneous clonal neoplastic bone marrow disorder that results in bone marrow failure due to ineffective hematopoiesis
•Associated with increased risk of progression to AML
•Marked by:
−Increased proliferation
−Increased apoptosis

84
Q

What is 5q syndrome?

A 
•Isolated deletion of chromosome 5q
•Female predominance
•Characterized mainly by anemia
−Thrombocytosis is common
−Neutropenia is mild
•Low rate of progression to AML
85
Q

What improves hemoglobin in MDS patients?

A

EPO

or EPO+G-CSF or darbepoetin

86
Q

How should MDS be treated?

A

If possible, allo transplant
If not + dz is stable, supportive care and investigational therapy with low toxicity
If not possible + dz is progressing, try lenalidomide, azacitidine, decitabine, or AML induction
In 5q syndrome, use lenalidomide

87
Q

What are the 3 sources of stem cells utilized in SCT?

A

umbilical cord blood
bone marrow
peripheral blood

88
Q

How is peripheral blood used in SCT?

A

Stem cells (defined by expression of CD34+) collected peripherally using apheresis (cell separator machine)
•Less invasive; less discomfort; less morbidity than BM Outpatient procedure
PBSCT results in more rapid hematopoietic recovery than BM
No difference in treatment outcome
–Using cytokine stimulation (G-CSF injections)
– BM releases large number CD34 stem cells into circulation
–Stem cells harvested via peripheral line

89
Q

What are some possible rate-limiting steps in cancer development?

A

alterations that increase the rate of cell division (c-myc activation, inactivation of Rb)
alterations that decrease genomic stability (p53, inactivation of mismatch repair genes)

90
Q

What is c-myc?

A

proto-oncogene that leads to cell division

typically activated by gene translocation

91
Q

What is a proto-oncogene?

A

Proto-oncogenes promote cancer when malignantly activated
–An activated proto-oncogene contributes to tumorigenesis by “gain-of-function”
–Thus, an activated proto-oncogene is genetically dominant at the cellular level
•an activated oncogene can elicit a new phenotype (tumorigenesis) even in the presence of the corresponding wildtype allele

92
Q

What is a tumor suppressor gene?

A

Tumor suppressor genes promote cancer when malignantly inactivated
–A tumor suppressor contributes to tumorigenesis by “loss-of-function”
–In most instances, an inactivated tumor suppressor gene is genetically recessive at the cellular level.
•it will not promote tumorigenesis in diploid cells unless the other (wildtype) allele is also lost or inactivated
•some exceptions:
–dominant-negative p53 mutations
–“haploinsufficient” tumor suppressor genes

93
Q

What percent of cancers are heritable?

A

Less than 10%

94
Q

What gene causes Familial retinoblastoma?

A

Rb

95
Q

What gene causes Li Fraumeni?

A

p53

96
Q

What gene causes Familial adenomatous polyposis coli?

A

APC

97
Q

What are the differences between sporadic and heritable retinoblastoma?

A

Heritable has earlier onset (2 years vs 6 years)

Heritable typically affects both eyes (while sporadic is often 1 tumor)

98
Q

How is Rb mutation transmitted?

A

Mendelian dominant

99
Q

How many people who carry Rb mutation get retinoblastoma?

A

Everyone! Heavily penetrant

100
Q

Which chromosome is affected in retinoblastoma?

A

13q14

101
Q

Which cancers are Rb associated with?

A

Always develop retinoblastoma

Sometimes develop osteosarcoma (low penentrance)

102
Q

What is the pathophys of Rb inactivation and cancer?

A

•hypophosphorylated Rb serves to restrain the proliferation of normal cells.
•regulated phosphorylation of Rb allows normal cells to proliferate at the correct time and place.
Inactivation of the Rb pathway occurs in most, if not all, human tumors
tumor suppressor gene

103
Q

What is different about heritable p53 mutations?

A

They are dominant negative (aka dominant tumor suppressor)

104
Q

What is the pathophys of p53 mutation and cancer?

A

induces G1 arrest
induces apoptosis in thymocytes
replication of damaged DNA ceases
inactivation of p53 occurs in most human tumors

105
Q

What is the most commonly mutated proto-oncogene in human tumors?

A

RAS

106
Q

What are characteristics of benign tumors?

A

Usually small
well-circumscribed
Closely resembles tissue of origin
Does not invad

107
Q

What are characteristics of malignant tumors?

A

all different sizes
poorly circumscribed
range from well to poorly differentiated
capable of invasion and metastasis

108
Q

What are the cytologic features of malignancy?

A
  1. high N:C ratio
  2. nuclear hyperchromatism
  3. prominent nucleoli
  4. nuclear pleomorphism
  5. high mitotic rate
109
Q

What is desmoplasia?

A

as malignant tumors grow, the neoplastic cells incite a local fibroblastic response in the primary site as it invades, as well as in the DISTANT site of metastasis.

The tissue attempts to “contain” the tumor growth at these sites

110
Q

What typically mets to the bone?

A

breast, prostate, renal cell ca

111
Q

Where does gastric cancer typically metastasize?

A 
Virchow's node (supraclavicular node)
Krukenberg tumor (bilateral ovarian mets)
112
Q

What is MM?

A

Multiple myeloma (MM) is characterized by the neoplastic proliferation of a single clone of plasma cells producing a monoclonal immunoglobulin. These plasma cells can damage bones, kidneys and hematopoiesis.

113
Q

What does a plasma cell come from?

A

B lymphocyte

Produces antibodies

114
Q

What are histopath signs of multiple myeloma?

A

fried egg appearance, Clock face chromatin and intracytoplasmic inclusions containing immunoglobin

115
Q

What is a Mott cell?

A

cytoplasm filled with Russel bodies
looks like a bunch of grapes
Not diagnostic for multiple myeloma, but may be seen

116
Q

What are the different parts of the immunoglobulin?

A

Heavy chain is the bottom of the T, light chain is the top

variable region on the ends

117
Q

Which immunoglobulins are important in multiple myeloma?

A

IgM and IgG

118
Q

Who gets multiple myeloma?

A

2x more common in African American
AA>white>Asian
More men than women (not hugely more frequent)
median age is 65 years old

119
Q

How does multiple myeloma present?

A
  • Anemia - 73 percent
  • Bone pain - 58 percent
  • Elevated creatinine - 48 percent
  • Hypercalcemia- 28 percent
  • Fatigue/generalized weakness - 32 percent
  • Weight loss - 24 percent, one-half of whom had lost 9+ kg
120
Q

How can multiple myeloma be diagnosed?

A
  1. bone marrow histology
  2. monoclonal immunoglobulins in serum and urine
  3. x-ray, CT (up to 80% of patients will have bone involvement)
121
Q

When is multiple myeloma treated?

A

MCRAB
•Anemia (hemoglobin <10 g/dL or 2 g/dL below normal)
•Hypercalcemia (serum calcium >11.5 mg/dL)
•Renal insufficiency (serum creatinine>2 mg/dL)
•Lytic bone lesions or severe osteopenia
•Extramedullary plasmacytoma

122
Q

When is thalidomide used?

A

multiple myeloma

123
Q

What is a risk of treatment of multiple myeloma?

A
  1. Proteosome inhibitors increase risk of shingles
    Need to prescribe acyclovir as prophylaxis along with treatment
  2. Thalidomide and lenalidomide increase risk of DVTs - need thromboprophylaxis
124
Q

How does multiple myeloma affect the bones?

A

Increases osteoclasts

Decreases osteoblasts

125
Q

What are bisphosphonates used for in cancer treatment?

A

Osteoclast inhibition

Used in multiple myeloma

126
Q

What is MGUS?

A

Less than 10% plasma cells in bone marrow
No end-organ damage
1% risk per year of conversion to multiple myeloma

127
Q

What is smoldering multiple myeloma?

A

10-60% plasma cells in bone marrow
No end-organ damage
10% risk of conversion to multiple myeloma

128
Q

What is MALT?

A

Extra-nodal Marginal Zone Lymphoma

129
Q

What is lymphoplasmacytic lymphoma?

A 
•Waldenstrӧm’s macroglobulinemia
–Secrete IgM
•Hyperviscosity
•Visual Impairment
•Neurologic problems
•Bleeding
•Cryoglobulinemia
•Morphology
–Plasmacytoid
–PAS staining Ig inclusions=Russell and Dutcher bodies
130
Q

Which indolent lymphomas are CD5+?

A

mantle zone

CLL/SLL

131
Q

Which indolent lymphoma is CD10+?

A

follicular

132
Q

Which indolent lymphomas are CD23+?

A

follicular

CLL/SLL

133
Q

Which indolent lymphoma is cyclin D1+?

A

mantle zone

134
Q

Which indolent lymphoma is IgM+?

A

lymphoplasmacytic lymphoma

135
Q

How does CML present?

A 
Median age 45-55
Symptoms:
•Fatigue
•Abdominal fullness
•Fever, chills, sweats, weight loss
Physical Findings:
•Hepatosplenomegaly
•Ecchymoses
Common Laboratory Findings:
•Increased mature and immature myeloid cells
•Basophilia
•Anemia
•Thrombocytosis
136
Q

What is seen on CML blood smear?

A

predominance of mature myeloid cells

+ some immature progenitors

137
Q

What is seen on CML bone marrow?

A

Hypercellular (less fat)

Increased myeloid:erythroid ratio

138
Q

What genetic changes are seen in CML?

A

BCR-ABL

Philadelphia chromosome

139
Q

What are the phases of CML?

A 
chronic phase (4-6 years)
accelerated phase (variable)
blast crisis (median survival 3-6 months)
140
Q

How is CML treated?

A

Imatinib

allogenic stem cell transplant

141
Q

How does imatinib work?

A

Imatinib sits in the ATP binding site of ABL (and c-kit) and prevents the transfer of phosphate groups to other proteins, hence blocking downstream signaling.
Metabolized by CYP450

142
Q

What are the side effects of imatinib?

A

bone marrow suppression (leukopenia, anemia, thrombocytopenia)
GI (nausea, vomiting, diarrhea)

143
Q

What can cause CML treatment to fail?

A

Development of new mutation

Increase in BCR-ABL gene copy number

144
Q

When should imatinib be used?

A

CML
Ph+ ALL
c-kit disease (GIST)

145
Q

How does polycythemia vera present?

A

•Elevated HGB/HCT on laboratory testing
•Thrombosis
•Symptoms:
–Pruritus (itching)-especially after showering
–Plethora (ruddy complexion)
–Abdominal fullness/early satiety (splenomegaly)

146
Q

What mutation is found in polycythemia vera?

A

JAK2 V617F

147
Q

What is seen in the bone marrow in polycythemia vera?

A 
•Moderate to marked hypercellularity
•Increased hematopoiesis
–Maturation intact
–Large clustered megakaryocytes
•Decreased / absent iron stores
•Increased reticulin fibers in minority of cases
148
Q

How is polycythemia vera diagnosed?

A
  1. Hgb 16.5+ g/dL in men or 16.0+ g/dL in women or HCT 49+% in men or 48+% in women or increased red cell mass
  2. Bone marrow biopsy showing hypercellularity for age with panmyelosis with pleomorphic mature megakaryocytes
  3. Presence of Jak2V617F or Jak2 exon 12 mutation
    Minor Criterion: Low serum erythropoietin, Endogenous erythroid colony formation in vitro
    Diagnosis requires either all major criteria or Major Criteria 1 and 2 and minor criterion
149
Q

What does polycythemia vera look like?

A

•Chronic Hypoxia (Altitude, cigarettes, sleep apnea, pulmonary disease)
•Erythropoietin abuse
•Familial erythrocytosis
–Mutation in Epo R

150
Q

What is the progression of polycythemia vera?

A 
Overall mortality 3% per year
–Thrombosis
•Increased by age 65+ and prior thrombosis
–Bleeding
–Transformation to AML
•Influenced by duration of disease
–Myelofibrosis (“Spent Phase”)
•More common in age 70+
151
Q

How is polycythemia vera treated?

A

•Therapeutic phlebotomy to maintain hct under 45, but avoiding iron deficiency
•Low dose aspirin (unless contraindicated)
•Aggressive management of reversible thrombotic risk factors (e.g. smoking, HTN)
•Consider cytoreduction if
–Patient is intolerant of phlebotomy
–Thrombocytosis develops
–Symptomatic or progressive splenomegaly
•Choice of cytoreductive therapy
–Less than age 40: interferon alfa
–More than age 40: hydroxyurea

152
Q

How does essential thrombocythemia present?

A

•Elevated platelets on laboratory testing
•Thrombosis
•Symptoms:
–Pruritus (itching)-especially after showering
–Erythromelalgia (severe pain in hands/feet)
–Abdominal fullness/early satiety (splenomegaly)

153
Q

What is the difference between essential thrombocytheima and polycythemia vera?

A

Jak2 V617F mutation found in 50% of patients with essential thrombocytosis
Allele burden may explain difference between PV and ET 5% of ET patients have mpl point mutations

154
Q

How is essential thrombocythemia diagnosed?

A

Major Criteria
1. Sustained platelet count 450,000+/mcl for at least 2 months
2. Bone marrow showing primarily megakaryocytic proliferation and panmyelosis with very rarely minor increase in reticulin fibers (grade 1)
3. No evidence of WHO diagnosis of polycythemia vera, primary myelofibrosis, CML (bcr-abl translocation), MDS or other myeloid neoplasms
4. Jak2, CALR or MPL
Minor Criterion
Presence other clonal marker OR in the absence of a clonal marker, no evidence of reactive thrombocytosis
All 4 major criteria need to be present or major criteria 1-3 and minor criterion for the diagnosis

155
Q

What is the clinical course of essential thrombocythemia?

A

•Uncertain if ET compromises life span
•Thrombosis
–Higher in patients over age 60, with a previous history of thrombosis, cardiovascular risk factors, JAK2V617F mutation
•Bleeding-Higher in patients with plt 1,500,000+/mcl
•Myelofibrosis (4-8% at 10 years)
•AML (1% in untreated patients)

156
Q

How is essential thrombocythemia managed?

A

•All patients: Aggressive management of cardiovascular risk factors
•Low risk: (Age <60, no high risk features)
–Low dose aspirin
•High risk (Age >60, prior thrombosis, cardiovascular risk factors)
–Low dose aspirin plus hydroxyurea
–Consider anagrelide or interferon alfa if hydroxyurea intolerant

157
Q

How does primary myelofibrosis present?

A 
•Splenomegaly
–Due to extramedullary hematopoiesis
–Abdominal fullness
•Symptoms of anemia
–Fatigue, pallor, dyspnea on exertion
•Bleeding/bruising
•Infection
•Cachexia
158
Q

What does primary myelofibrosis look like on histopath?

A 
Teardrop RBCs
leukoerythroblastic features (blood looks like marrow)
159
Q

What does bone marrow of primary myelofibrosis look like?

A

•Hypercellular; Megakaryocytic hyperplasia
•Pleomorphism of megakaryocytes
•Granulocytic / erythroid maturation intact
•Fibrotic phase - loss of hematopoiesis
–Megakaryocytes spared
•Osteosclerosis
–Increased osteoclasts and osteoblasts

160
Q

What is the clinical progression of primary myelofibrosis?

A

Variable life expectancy (months to decades)

Progression to AML in 10-15%

161
Q

How is primary myelofibrosis treated?

A 
•Supportive Measures
–Erythropoietin/transfusions
–Hydroxyurea to control SBC
–Splenectomy/splenic radiation
•Active Therapy
–Stem cell transplant
–Thalidomide/Corticosteroids
–Jak inhibitors
162
Q

What is peripheral T cell lymphoma?

A
  • Rare – make up 5-10% of lymphomas in US
  • More common in Asia
  • No standard treatment regimens
  • Range from indolent to highly aggressive
  • Generally worse prognosis than their B-cell counter parts
163
Q

What is peripheral T cell lymphoma NOS (not otherwise specified)?

A 
•Waste-basket diagnosis
•Often present with rash
•Uniformly poor prognosis
•No standard treatment regimens
•Morphology &amp;
Immunohistochemistry:
– TCR rearrangements
–Loss of T-cell surface markers
–No specific cytogenetic abnormalities
164
Q

What is anaplastic large cell T cell lymphoma?

A

type of T cell lymphoma
ALK + patients are younger with better prognosis
ALK - patients are older with worse prognosis
Both are CD30+ and show horseshoe-shaped nuclei

165
Q

What is mycosis fungoides?

A 
Cutaneous T cell lymphoma
•Indolent lymphoma
–Delayed diagnosis
–History of eczema
•Skin lesions can progress
–patch-plaque stage
–Tumor
–Lymph node involvement
–Sezary Syndrome
•Systemic involvement
•Peripheral blood findings
166
Q

How is mycosis fungoides treated?

A 
–topical glucocorticoids
–XRT
–PUVA
–Topical chemotherapy
–Phototherapy
–Photopheresis
–Retinoids
–HDAC inhibitors
–Chemotherapy
–Transplant
167
Q

What is adult T cell leukemia/lymphoma?

A 
•Driven by HTLV-1
•Transmitted by breastfeeding, blood transfusions, needle sharing and sexual intercourse.
•Long latency of 10-30 years.
•Morphology: Flower Cell
•Immunohistochemistry:
–TAX (+)
–+CD4, +CD25, occasionally +CD30
Very aggressive
168
Q

What is seen on histopath in adult T cell leukemia/lymphoma?

A

flower cell

169
Q

How does HTLV-1 ALL present?

A

lymphadenopathy
rash
hypercalcemia
CNS involvement

170
Q

How does brentuximab vedotin work?

A 
very potent
potent antimicrotubule agent
protease-cleavable linker
anti-CD30 monoclonal antibody
ADC binds to CD30
MMAE disrupts microtubule network
ADC-CD30 complex traffics to lysosome
MMAE is released
Apoptosis
G2/M cell cycle arrest
171
Q

What expresses CD30?

A

hodgkin lymphoma

systemic anaplastic large cell lymphoma

172
Q

What is Hodgkin lymphoma?

A

Lymphoid neoplasm defined by presence of Reed-Sternberg cells
CD30+
spreads in a contiguous pattern

173
Q

How does Hodgkin lymphoma present?

A 
•Large mediastinal mass
•B-symptoms:
–Pruritis
–Pel Ebstein fevers
–Painful nodes with alcohol consumption
174
Q

How is Hodgkin lymphoma treated?

A 
ABVD every 2 weeks
•Adriamycin
•Bleomycin
•Vinblastine
•Dacarbazine
First need to evaluate Cardiac and Lung function
–ECHO
–PFTs with DLCO
Number of cycles given depends partly on the stage and Prognosis Score
–Patients may get 2-6 cycles
•Highly Emetogenic
•Not all patients lose their hair
Radiation also used
Can also use stem cell transplant in refractory cases
175
Q

What new agents can be used in Hodgkins?

A

brentuximab

nivolumab (anti-PD1)

Oncology (3 decks)

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