Heme-Onc Part 6

Front 1

immunohistochemistry ~~

Back 1

CD markers

Front 2

working formulation ~~

Back 2

*clinical* behavior

Front 3

low grade :

(2)

Back 3

1. slow, indolent course

2. incurable

Front 4

intermediate grade:

(2)

Back 4

1. more acute presentation than low grade

2. some curable

Front 5

high grade:

(3)

Back 5

1. acute presentation with high risk of death

2. but curable

3. ~ masses

Front 6

which two lymphomas do NOT fall under the WF?

Back 6

SLL,

Hodgkins

Front 7

B-cell entry into LN's requires:

Back 7

antigen presentation

Front 8

class switching =

Back 8

IgM to IgG, D, or A

Front 9

class switching and hypersomatic mutations occur in:

Back 9

the germinal center

Front 10

plasma cells migrate back to:

Back 10

the marrow

Front 11

**lymphomas are ____________ diseases**

Back 11

clonal

- express EITHER kappa of lambda

- all the abnormal lymphocytes have the SAME Ig

Front 12

3 risk factors for lymphoma:

Back 12

1. age

2. AI, inf, transplant

3. certain viruses

Front 13

3 viruses that increase risk of lymphoma:

Back 13

1. EBV

2. HIV

3. HepC

Front 14

lymphomas can form in any place where there are _____________________;

(4)

Back 14

lymphocytes;


LN's, marrow, mucosa, solid organs

Front 15

lymphomas often present with:

(2)

Back 15

1. systemic infection symps

2. symps due to mass/growth (e.g. satiety)

Front 16

2 high-grade lymphomas:

Back 16

1. Burkitt's

2. Lymphoblastic Lymphoma

Front 17

what does overexpression of MYC result in?

(2)

Back 17

1. extremely high cell proliferation

2. inc. LDH proliferation

Front 18

Burkitt's: no symptoms >2 years past treatment =

Back 18

cured

Front 19

3 types of Burkitt's:

Back 19

1. endemic

2. sporadic (outside malaria belt)

3. HIV-associated

Front 20

endemic Burkitt's ~~

(3)

Back 20

1. EBV

2. malaria

3. kids

Front 21

Burkitt's: starry sky histology =

Back 21

histiocytes gobbling up cell debris, with lots of white vacuoles in them

Front 22

histiocytes =

Back 22

stationary macrophages

Front 23

b/c T cells mature in the thymus, a T-cell lymphoma like LL can often result in:

Back 23

tumors of the anterior medistinum

Front 24

if >20% of a sample's cells are immature T-cells, the LL diagnosis becomes:

Back 24

ALL

Front 25

like ALL, LL has:

(2)

Back 25

CNS, testicular involvement

Front 26

stage and LDH levels do NOT ___________________________ in LL

Back 26

affect prognosis

Front 27

DLCL ~ single mass, but cells are:

Back 27

spread throughout sample

Front 28

BCL6 is normally _____

Back 28

off

- cells not arrested;

- when on, represses transcription,
keeping cells in areas that allow for more mutations

Front 29

in DLCL, relapse after remission is:

Back 29

uncommmon

Front 30

3 types of DLCL, even though cells look exactly the same histologically:

Back 30

1. Germinal Center B-cell - like

2. activated B-cell-like

3. (mediastinal)

Front 31

2 features of follicular lymphoma:

Back 31

1. also germinal-center-processed

2. may progress to DLCL

Front 32

***what does BCL2 do?***

Back 32

*inhibits* apoptosis

Front 33

cytometry of follicular lymphoma:

Back 33

ALL kappa,

NO lambda

Front 34

follicular lymphoma relapses can occur:

Back 34

>10 years later

Front 35

Small Lymphocytic Lymphoma =

Back 35

***LN version of CLL***

- e/t about CLL can be applied to SLL

Front 36

***SLL on flow:***

(2)

Back 36

CD5+

CD23+

Front 37

Reed-Sternberg cells =

Back 37

abnormally-activated B cells

Front 38

Hodgkins Lymphoma cells are also:

Back 38

germinal center cells, though many B-markers are NOT expressed

Front 39

like follicular lymphomas, Hodgkins can:

Back 39

re-appear >10 years later

Front 40

3 signs of lymphoma:

Back 40

1. incidental finding

2. mass/growth effects

3. B-symptoms

Front 41

3 B-symptoms:

Back 41

1. weight loss >10% in 6 months

2. drenching night sweats

3. fever >38 C

Front 42

"reactive" LN ~~

Back 42

infection

Front 43

2 features of reactive LN's:

Back 43

1. grow quickly

2. painful/tender

Front 44

when to biopsy a LN:

(4)

Back 44

1. >2 cm

2. slow-growing

3. painLESS

4. pts have risk factors for lymphoma

Front 45

Stage I =

Back 45

single site

Front 46

Stage II =

Back 46

2 or more sites on SAME SIDE of diaphragm

Front 47

Stage III =

Back 47

2 or more sites on OPPOSITE side of diaphragm

Front 48

Stage IV =

Back 48

liver or bone involvement

Front 49

"E" is added to lymphoma staging if:

Back 49

extranodal sites are involved

Front 50

B is added to lymphoma stage if:

Back 50

ANY of the B-symps are occurring

Front 51

B symps in lymphoma ~~

Back 51

worse prognosis

Front 52

international prognostic index ~~ to ____________ ONLY

Back 52

DLCL

Front 53

IPI gives one point for each of the following:

(5)

Back 53

1. > nl LDH

2. > 60 y.o.

3. Stage III or IV DLCL

4. >1 extranodal site

5. performance status > or equal to 2

Front 54

ECOG performance status: 0 =

(2)

Back 54

asymp and fully active

Front 55

ECOG performance status: 1 =

(2)

Back 55

symptomatic but completely active

Front 56

ECOG performance status: 2 =

(3)

Back 56

symp,

<50% of waking hours in bed,

able to do self-care

Front 57

ECOG performance status: 3 =

(3)

Back 57

symp,

>50% of waking hour in bed,

limited self-care

Front 58

ECOG performance status: 4 =

(3)

Back 58

bed-bound,

completely disabled,

NO self-care

Front 59

ECOG tally: 0 points =

Back 59

4-year survival rate of 95%

Front 60

ECOG tally: 1-2 points =

Back 60

4-year survival rate of 80%

Front 61

ECOG tally: 3-5 points =

Back 61

4-year survival rate of 55%

Front 62

treatment of lymphomas: palliative =

Back 62

chemo and Rx

Front 63

intense chemo for lymphomas =

Back 63

chemo followed by autologous BMT,

reserved for those with curative diseases who've relapsed

Front 64

more Rx therapy is used for:

Back 64

EARLY stages of lymphoma,

more chemo for late

- but combos often used

Front 65

follicles are normally located at:

Back 65

the periphery

Front 66

low grades ~~ advanced stages, due to:

Back 66

pts being asymp for so long

Front 67

multiple gene mutations in a lymphoma =>

Back 67

worse prognosis

Front 68

normal LN's have a mix of:

Back 68

B-cells and T-cells

Front 69

parafollicular hyperplasia =

Back 69

increase in T-cells due to infection

Front 70

follicular hyperplasia =

Back 70

proliferation of B-cells due to inc. need for AB production

=> **enlargement of germinal centers**

Front 71

histiocytosis =

Back 71

proliferation of macrophages due to stimulation of antigen-presentation cells

=> **expansion of medullary sinuses**

Front 72

flow is useless for Hodgkins b/c:

Back 72

sample is mostly *normal* cells

Front 73

**Flow is useful for _____________ too**

Back 73

lymphomas

Front 74

germinal centers should be on the:

Back 74

periphery

Front 75

SLL = _________ form of CLL

Back 75

tissue/LN form of CLL

Front 76

Low-grade lymphomas can become high-grade lymphomas =>

Back 76

sometimes need to treat low-grade (despite no symptoms), if indicated that it’ll progress

Front 77

2 classic causes of mediastinal masses:

Back 77

1. Hodgkins

2. T-cell lymphocytosis

Front 78

Teenager and chronic symptoms =

Back 78

Hodgkins

Front 79

Teenager and acute symptoms =

Back 79

T-cell ALL

Front 80

***enlarged supraclavicular LN's ~~**

Back 80

cancer

Front 81

B cells are much more common than:

Back 81

T cell lymphomas,

and DLCL is the most common kind.

Front 82

plasma cell dyscrasia =

Back 82

plasma cell cancer

Front 83

multiple myeloma =

Back 83

cancer of plasma cells in which abnormal ones (called myeloma cells) produce an overabundance of M protein back at the marrow

(i.e. malignancy occurs *after* germinal center processing)

Front 84

3 M protein features:

Back 84

1. a monoclonal AB

2. does NOT bind to anything => NOT autoreactive

3. usually IgG or IgA

Front 85

biggest risk factor for myeloma =

Back 85

age

>50 y.o., think myeloma

- won't happen <50

Front 86

myeloma produces a gamma band on SPEP; will tell you:

Back 86

there are more AB's than normal, but NOT which ones

=> need to show inc. in *monoclonal* AB

=> IEF automatically

- if it shows kappa or lambda only, it's proof of monoclonal ==> myeloma

Front 87

myelomas also continually produce:

Back 87

light chains, more and more as time goes on

Front 88

myeloma ~~ bone disease, due to:

Back 88

increase in osteoclast activity (via RANKL) and lack of response of osteoblasts

=> dissolution of bone/lytic lesions

=> hypercalcemia, fractures

Front 89

osteoblasts ________ bone, osteoclasts __________ it

Back 89

form;

erode

Front 90

bone lesions from myeloma are ________________ on bone scans

Back 90

*undetectable*

- due to dec. osteoblast response

Front 91

hypercalcemia => ______________ symptoms

Back 91

non-specific

Front 92

anemia is common in myeloma, but it's NOT:

Back 92

hemolytic

- normocytic, normochromic

Front 93

effects of M protein:

(3)

Back 93

1. ***renal failure***

2. hypogammaglobulinemia

3. hypersensitivity (minor)

Front 94

hypogammaglobulinemia =>

Back 94

infections, especially of encapsulated bact.

Front 95

4 lab effects seen in myeloma:

Back 95

1. inc. protein:albumin ratio

2. dec. anion gap

3. **rouleoux** formation

4. inc. ESR

Front 96

2 things **required** to diagnose active multiple myeloma:

Back 96

1. serum M protein > or equal to 3 OR > or equal to 10% of marrow is clonal plasma cells

2. at least ONE of CRAB

Front 97

CRAB =

Back 97

(hyper)Calcemia (>11.5)

Renal insufficiency (creatinine > 1.73)

Anemia

Bone Disease

order tests for each one to rule them out

Front 98

if no CRAB and only >3/10, you have:

Back 98

asymptomatic myeloma

Front 99

because the prognosis of myeloma is extremely variable, there are 3 categories to tell pts about:

Back 99

1. standard risk (8-10 yrs median survival)

2. intermediate risk (4-5 yrs)

3. high risk (3 yrs)

Front 100

risk/prognosis of *individual* with myeloma is calculated via:

(2)

Back 100

1. *cytogenetics*

2. profile factures

Front 101

4 features of myeloma treatment:

Back 101

1. incurable

2. getting better at prolonging life

3. multiple combos of multiple drugs

4. eventually pts die of complications of disease OR therapy

Front 102

3 drugs used in combo to treat myeloma:

Back 102

1. steroids

2. cytotoxic drugs

3. proteosome inhibitors

Front 103

chemo "maintenance" =

Back 103

lower doses of continuous therapy

Front 104

Monoclonal Gammopathy of Unknown Sig =

Back 104

proliferation of plasma cells *without* symptoms

i.e. <3/10 but asymp

Front 105

MGUS = most common cause of:

Back 105

increase in M. protein

Front 106

MGUS is a ______________________ condition

Back 106

pre-maliignant

==> myeloma at the rate of 1.5% inc. in risk per year

(MGUS is the first hit towards myeloma)

Front 107

(asymp myeloma to active multiple myeloma occurs at the rate of:

Back 107

10% inc. in risk per year)

Front 108

treatment for MGUS:

Back 108

no treatment, just watch

- multistep, just like Vogelgram

Front 109

amyloidosis =

Back 109

sheets of fibrils, seen via histopathology

- diagnosis *req's* biopsy

Front 110

amyloid fibrils are deposited in:

Back 110

renal (esp with AA amyloidosis),

cardiac,

PNS,

other tissue (e.g. fat)

blood (=> mucocutaneous bleeding)

Front 111

amyloidosis can be either:

Back 111

symp. OR asymp.

Front 112

amount of M protein resulting in symptoms is MUCH smaller in amyloidosis than in:

Back 112

myelomas

- i.e. amyloidosis = <3/<10%

Front 113

AL amyloidosis =

Back 113

bad luck MGUS

Front 114

in order to receive treatment for AL amyloidosis, you need:

(2)

Back 114

1. symptoms DUE to amyloid

2. evidence that M protein caused it

Front 115

treatment of AL amyloidosis is similar to myeloma - use:

Back 115

steroids, cytotoxic drugs, proteosome inhibitors

- but prognosis is WORSE

Front 116

(even in myeloma, you can see:

Back 116

amyloid)

Front 117

Waldenstrom's Macroglobulinemia =

Back 117

cancer of B cells that produce IgM

Front 118

*WM looks like:

(2)

Back 118

1. low-grade lymphoma

2. plasma cell cancer

=> lymphoplasmacytic lymphoma

Front 119

symptoms of WM:

(3)

Back 119

1. B symps

2. hepato/spleno

3. hyperviscosity

Front 120

the hyperviscosity of WM is a result of:

Back 120

large IgM tetramer

=> neurologic symptoms, HF, distinctive retinopathy

Front 121

the distinctive retinopathy of WM is referring to:

Back 121

sausageing of retinal vessels (where both the arterioles and the venules are dilated, except at where they cross

Front 122

sausiging retinal vessles =>

(2)

Back 122

1. optic disc edema

2. retinal hemorrhage

Front 123

treatment for hyperviscosity =

Back 123

plasmaphoresis

Front 124

also distinctive of WM: the IgM is produced by cells that have NOT:

Back 124

undergone Ig class switching

=> some are *autoreactive*

=> rxns against RC's, myelin, and IgG

Front 125

autoreactive rxn of WM plasma cells against RC's =>

Back 125

cold-like agglutination

Front 126

diagnosis of WM =

Back 126

IgM monoclonal gammopathy of any size

AND

> or equal to 10% of marrow is made up of lymphoplasmacytic lymphocytes that are positive for IgM, CD20, and CD19

Front 127

treatment of WM =

Back 127

same as for low-grade lymphomas, esp. Rituximab

Front 128

leading kids cancer =

Back 128

ALL

Front 129

AML + neutropenia or fever or inf =

Back 129

emergency,

esp. if ANC <500

Front 130

Wilm's tumore = most common:

Back 130

kidney tumor

- **highly curable**

Front 131

Ewing's sarcoma =

Back 131

bone tumor

Front 132

rhabdomyosarcoma ~~

(2)

Back 132

1. proptosis

2. vaginal mass

Front 133

XRT =

Back 133

external beam Rx,

given in small amount over time

Front 134

when you see these 3 things, you need to order an SPEP/IEF: patient over 50 with:

Back 134

1. a pathologic fracture, lytic bone lesion, or hypercalcemia

2. normochromic, normocytic anemia,

3. new onset (acute) renal failure,

Front 135

what are the 4 lymphocytes?

Back 135

B, T, NK, and plasma cells

- otherwise: granulocytes, plat's, and monocytes

Front 136

T-cell markers are:

Back 136

CD 1- 8

Front 137

definitive flow for APL =

Back 137

CD34 NEG, HLADR NEG

(other AML's are typically positive for both)

Front 138

myeloblasts are:

Back 138

large, immature cells with multiple nuclei

Front 139

**3 features of benign neutrophilia:**

Back 139

1. left shift, inc. M/E ratio are not as pronounced as in CML

2. **normal** basophil count

3. normal karyotype

Front 140

what are lymphblasts?

Back 140

large, immature lymphoid cells with high N:C (little cytoplasm) and single nucleolus

Front 141

atypical lymphocytosis is the result of:

Back 141

mononucleosis,

other viral inf's

Front 142

clinical presentation of hairy cell leukemia:

(3)

Back 142

1. **males** (9:1)

2. massive splenomegaly

3. pancytopenia

Front 143

symptoms of multiple myeloma are the result of:

Back 143

1. mass/growth

2. secretions of malignant plasma cells
(M prot, light chains)

Front 144

B2 microglobulin and albumin are useful for ____________ multiple myeloma

Back 144

staging

Front 145

***kapp/lambda staining is:***

Back 145

BLUE

- blue on both = polyclonal = NOT myeloma

Front 146

renal dysfunction in multiple myeloma appears as:

(3)

Back 146

1. Bence Jones protein in urine

2. light chain nephropathy

3. amyloid in the glomeruli and blood vessles

Front 147

Bence Jones protein =

Back 147

Ig light chain found in urine

Front 148

lytic lesion =

Back 148

destruction of an area of bone

Front 149

radiculopathy =

Back 149

nerve root compression,

as seen in AMM

Front 150

AL Amyloidosis is associated with:

(2)

Back 150

1. mult myeloma

2. abnormal light chains

Front 151

clinical presentation of AL Amyloidosis:

(2)

Back 151

1. NO classic presentation

2. kidney is most often affected
(followed by liver, heart, neuro)

Front 152

diagnostic for AL Amyloidosis =

Back 152

positive Congo red stain

=> apple green when polarized

Front 153

features of WM:

(

Back 153

1. adult disease

2. ~~ mature, monoclonal B cells

Front 154

classic presentation of WM =

(3)

Back 154

1. HA

2. blurred vision

3. hyperviscosity

Front 155

diagnostic of WM =

(2)

Back 155

SPEP/IEF showing abnormal amount of IgM;

serum viscosity

Front 156

marrow of WM =

Back 156

hypercellular, with NO fat

Front 157

cancer-promoting factors:

(5)

Back 157

1. obesity

2. red/processed meat

3. sat. fat

4. alcohol

5. aflatoxins

Front 158

cancer risk-reducing factors:

(3)

Back 158

1. fruits/veggies/beans

2. healthy body weight

3. daily exercise

Front 159

aflatoxin =

Back 159

type of mycotoxin produced by Aspergillus

- commonly conatminates legumes/peanuts