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159 Cards in this Set
- Front
- Back
immunohistochemistry ~~
|
CD markers
|
|
working formulation ~~
|
*clinical* behavior
|
|
low grade :
(2) |
1. slow, indolent course
2. incurable |
|
intermediate grade:
(2) |
1. more acute presentation than low grade
2. some curable |
|
high grade:
(3) |
1. acute presentation with high risk of death
2. but curable 3. ~ masses |
|
which two lymphomas do NOT fall under the WF?
|
SLL,
Hodgkins |
|
B-cell entry into LN's requires:
|
antigen presentation
|
|
class switching =
|
IgM to IgG, D, or A
|
|
class switching and hypersomatic mutations occur in:
|
the germinal center
|
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plasma cells migrate back to:
|
the marrow
|
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**lymphomas are ____________ diseases**
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clonal
- express EITHER kappa of lambda - all the abnormal lymphocytes have the SAME Ig |
|
3 risk factors for lymphoma:
|
1. age
2. AI, inf, transplant 3. certain viruses |
|
3 viruses that increase risk of lymphoma:
|
1. EBV
2. HIV 3. HepC |
|
lymphomas can form in any place where there are _____________________;
(4) |
lymphocytes;
LN's, marrow, mucosa, solid organs |
|
lymphomas often present with:
(2) |
1. systemic infection symps
2. symps due to mass/growth (e.g. satiety) |
|
2 high-grade lymphomas:
|
1. Burkitt's
2. Lymphoblastic Lymphoma |
|
what does overexpression of MYC result in?
(2) |
1. extremely high cell proliferation
2. inc. LDH proliferation |
|
Burkitt's: no symptoms >2 years past treatment =
|
cured
|
|
3 types of Burkitt's:
|
1. endemic
2. sporadic (outside malaria belt) 3. HIV-associated |
|
endemic Burkitt's ~~
(3) |
1. EBV
2. malaria 3. kids |
|
Burkitt's: starry sky histology =
|
histiocytes gobbling up cell debris, with lots of white vacuoles in them
|
|
histiocytes =
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stationary macrophages
|
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b/c T cells mature in the thymus, a T-cell lymphoma like LL can often result in:
|
tumors of the anterior medistinum
|
|
if >20% of a sample's cells are immature T-cells, the LL diagnosis becomes:
|
ALL
|
|
like ALL, LL has:
(2) |
CNS, testicular involvement
|
|
stage and LDH levels do NOT ___________________________ in LL
|
affect prognosis
|
|
DLCL ~ single mass, but cells are:
|
spread throughout sample
|
|
BCL6 is normally _____
|
off
- cells not arrested; - when on, represses transcription, keeping cells in areas that allow for more mutations |
|
in DLCL, relapse after remission is:
|
uncommmon
|
|
3 types of DLCL, even though cells look exactly the same histologically:
|
1. Germinal Center B-cell - like
2. activated B-cell-like 3. (mediastinal) |
|
2 features of follicular lymphoma:
|
1. also germinal-center-processed
2. may progress to DLCL |
|
***what does BCL2 do?***
|
*inhibits* apoptosis
|
|
cytometry of follicular lymphoma:
|
ALL kappa,
NO lambda |
|
follicular lymphoma relapses can occur:
|
>10 years later
|
|
Small Lymphocytic Lymphoma =
|
***LN version of CLL***
- e/t about CLL can be applied to SLL |
|
***SLL on flow:***
(2) |
CD5+
CD23+ |
|
Reed-Sternberg cells =
|
abnormally-activated B cells
|
|
Hodgkins Lymphoma cells are also:
|
germinal center cells, though many B-markers are NOT expressed
|
|
like follicular lymphomas, Hodgkins can:
|
re-appear >10 years later
|
|
3 signs of lymphoma:
|
1. incidental finding
2. mass/growth effects 3. B-symptoms |
|
3 B-symptoms:
|
1. weight loss >10% in 6 months
2. drenching night sweats 3. fever >38 C |
|
"reactive" LN ~~
|
infection
|
|
2 features of reactive LN's:
|
1. grow quickly
2. painful/tender |
|
when to biopsy a LN:
(4) |
1. >2 cm
2. slow-growing 3. painLESS 4. pts have risk factors for lymphoma |
|
Stage I =
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single site
|
|
Stage II =
|
2 or more sites on SAME SIDE of diaphragm
|
|
Stage III =
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2 or more sites on OPPOSITE side of diaphragm
|
|
Stage IV =
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liver or bone involvement
|
|
"E" is added to lymphoma staging if:
|
extranodal sites are involved
|
|
B is added to lymphoma stage if:
|
ANY of the B-symps are occurring
|
|
B symps in lymphoma ~~
|
worse prognosis
|
|
international prognostic index ~~ to ____________ ONLY
|
DLCL
|
|
IPI gives one point for each of the following:
(5) |
1. > nl LDH
2. > 60 y.o. 3. Stage III or IV DLCL 4. >1 extranodal site 5. performance status > or equal to 2 |
|
ECOG performance status: 0 =
(2) |
asymp and fully active
|
|
ECOG performance status: 1 =
(2) |
symptomatic but completely active
|
|
ECOG performance status: 2 =
(3) |
symp,
<50% of waking hours in bed, able to do self-care |
|
ECOG performance status: 3 =
(3) |
symp,
>50% of waking hour in bed, limited self-care |
|
ECOG performance status: 4 =
(3) |
bed-bound,
completely disabled, NO self-care |
|
ECOG tally: 0 points =
|
4-year survival rate of 95%
|
|
ECOG tally: 1-2 points =
|
4-year survival rate of 80%
|
|
ECOG tally: 3-5 points =
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4-year survival rate of 55%
|
|
treatment of lymphomas: palliative =
|
chemo and Rx
|
|
intense chemo for lymphomas =
|
chemo followed by autologous BMT,
reserved for those with curative diseases who've relapsed |
|
more Rx therapy is used for:
|
EARLY stages of lymphoma,
more chemo for late - but combos often used |
|
follicles are normally located at:
|
the periphery
|
|
low grades ~~ advanced stages, due to:
|
pts being asymp for so long
|
|
multiple gene mutations in a lymphoma =>
|
worse prognosis
|
|
normal LN's have a mix of:
|
B-cells and T-cells
|
|
parafollicular hyperplasia =
|
increase in T-cells due to infection
|
|
follicular hyperplasia =
|
proliferation of B-cells due to inc. need for AB production
=> **enlargement of germinal centers** |
|
histiocytosis =
|
proliferation of macrophages due to stimulation of antigen-presentation cells
=> **expansion of medullary sinuses** |
|
flow is useless for Hodgkins b/c:
|
sample is mostly *normal* cells
|
|
**Flow is useful for _____________ too**
|
lymphomas
|
|
germinal centers should be on the:
|
periphery
|
|
SLL = _________ form of CLL
|
tissue/LN form of CLL
|
|
Low-grade lymphomas can become high-grade lymphomas =>
|
sometimes need to treat low-grade (despite no symptoms), if indicated that it’ll progress
|
|
2 classic causes of mediastinal masses:
|
1. Hodgkins
2. T-cell lymphocytosis |
|
Teenager and chronic symptoms =
|
Hodgkins
|
|
Teenager and acute symptoms =
|
T-cell ALL
|
|
***enlarged supraclavicular LN's ~~**
|
cancer
|
|
B cells are much more common than:
|
T cell lymphomas,
and DLCL is the most common kind. |
|
plasma cell dyscrasia =
|
plasma cell cancer
|
|
multiple myeloma =
|
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) |
|
3 M protein features:
|
1. a monoclonal AB
2. does NOT bind to anything => NOT autoreactive 3. usually IgG or IgA |
|
biggest risk factor for myeloma =
|
age
>50 y.o., think myeloma - won't happen <50 |
|
myeloma produces a gamma band on SPEP; will tell you:
|
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 |
|
myelomas also continually produce:
|
light chains, more and more as time goes on
|
|
myeloma ~~ bone disease, due to:
|
increase in osteoclast activity (via RANKL) and lack of response of osteoblasts
=> dissolution of bone/lytic lesions => hypercalcemia, fractures |
|
osteoblasts ________ bone, osteoclasts __________ it
|
form;
erode |
|
bone lesions from myeloma are ________________ on bone scans
|
*undetectable*
- due to dec. osteoblast response |
|
hypercalcemia => ______________ symptoms
|
non-specific
|
|
anemia is common in myeloma, but it's NOT:
|
hemolytic
- normocytic, normochromic |
|
effects of M protein:
(3) |
1. ***renal failure***
2. hypogammaglobulinemia 3. hypersensitivity (minor) |
|
hypogammaglobulinemia =>
|
infections, especially of encapsulated bact.
|
|
4 lab effects seen in myeloma:
|
1. inc. protein:albumin ratio
2. dec. anion gap 3. **rouleoux** formation 4. inc. ESR |
|
2 things **required** to diagnose active multiple myeloma:
|
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 |
|
CRAB =
|
(hyper)Calcemia (>11.5)
Renal insufficiency (creatinine > 1.73) Anemia Bone Disease order tests for each one to rule them out |
|
if no CRAB and only >3/10, you have:
|
asymptomatic myeloma
|
|
because the prognosis of myeloma is extremely variable, there are 3 categories to tell pts about:
|
1. standard risk (8-10 yrs median survival)
2. intermediate risk (4-5 yrs) 3. high risk (3 yrs) |
|
risk/prognosis of *individual* with myeloma is calculated via:
(2) |
1. *cytogenetics*
2. profile factures |
|
4 features of myeloma treatment:
|
1. incurable
2. getting better at prolonging life 3. multiple combos of multiple drugs 4. eventually pts die of complications of disease OR therapy |
|
3 drugs used in combo to treat myeloma:
|
1. steroids
2. cytotoxic drugs 3. proteosome inhibitors |
|
chemo "maintenance" =
|
lower doses of continuous therapy
|
|
Monoclonal Gammopathy of Unknown Sig =
|
proliferation of plasma cells *without* symptoms
i.e. <3/10 but asymp |
|
MGUS = most common cause of:
|
increase in M. protein
|
|
MGUS is a ______________________ condition
|
pre-maliignant
==> myeloma at the rate of 1.5% inc. in risk per year (MGUS is the first hit towards myeloma) |
|
(asymp myeloma to active multiple myeloma occurs at the rate of:
|
10% inc. in risk per year)
|
|
treatment for MGUS:
|
no treatment, just watch
- multistep, just like Vogelgram |
|
amyloidosis =
|
sheets of fibrils, seen via histopathology
- diagnosis *req's* biopsy |
|
amyloid fibrils are deposited in:
|
renal (esp with AA amyloidosis),
cardiac, PNS, other tissue (e.g. fat) blood (=> mucocutaneous bleeding) |
|
amyloidosis can be either:
|
symp. OR asymp.
|
|
amount of M protein resulting in symptoms is MUCH smaller in amyloidosis than in:
|
myelomas
- i.e. amyloidosis = <3/<10% |
|
AL amyloidosis =
|
bad luck MGUS
|
|
in order to receive treatment for AL amyloidosis, you need:
(2) |
1. symptoms DUE to amyloid
2. evidence that M protein caused it |
|
treatment of AL amyloidosis is similar to myeloma - use:
|
steroids, cytotoxic drugs, proteosome inhibitors
- but prognosis is WORSE |
|
(even in myeloma, you can see:
|
amyloid)
|
|
Waldenstrom's Macroglobulinemia =
|
cancer of B cells that produce IgM
|
|
*WM looks like:
(2) |
1. low-grade lymphoma
2. plasma cell cancer => lymphoplasmacytic lymphoma |
|
symptoms of WM:
(3) |
1. B symps
2. hepato/spleno 3. hyperviscosity |
|
the hyperviscosity of WM is a result of:
|
large IgM tetramer
=> neurologic symptoms, HF, distinctive retinopathy |
|
the distinctive retinopathy of WM is referring to:
|
sausageing of retinal vessels (where both the arterioles and the venules are dilated, except at where they cross
|
|
sausiging retinal vessles =>
(2) |
1. optic disc edema
2. retinal hemorrhage |
|
treatment for hyperviscosity =
|
plasmaphoresis
|
|
also distinctive of WM: the IgM is produced by cells that have NOT:
|
undergone Ig class switching
=> some are *autoreactive* => rxns against RC's, myelin, and IgG |
|
autoreactive rxn of WM plasma cells against RC's =>
|
cold-like agglutination
|
|
diagnosis of WM =
|
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 |
|
treatment of WM =
|
same as for low-grade lymphomas, esp. Rituximab
|
|
leading kids cancer =
|
ALL
|
|
AML + neutropenia or fever or inf =
|
emergency,
esp. if ANC <500 |
|
Wilm's tumore = most common:
|
kidney tumor
- **highly curable** |
|
Ewing's sarcoma =
|
bone tumor
|
|
rhabdomyosarcoma ~~
(2) |
1. proptosis
2. vaginal mass |
|
XRT =
|
external beam Rx,
given in small amount over time |
|
when you see these 3 things, you need to order an SPEP/IEF: patient over 50 with:
|
1. a pathologic fracture, lytic bone lesion, or hypercalcemia
2. normochromic, normocytic anemia, 3. new onset (acute) renal failure, |
|
what are the 4 lymphocytes?
|
B, T, NK, and plasma cells
- otherwise: granulocytes, plat's, and monocytes |
|
T-cell markers are:
|
CD 1- 8
|
|
definitive flow for APL =
|
CD34 NEG, HLADR NEG
(other AML's are typically positive for both) |
|
myeloblasts are:
|
large, immature cells with multiple nuclei
|
|
**3 features of benign neutrophilia:**
|
1. left shift, inc. M/E ratio are not as pronounced as in CML
2. **normal** basophil count 3. normal karyotype |
|
what are lymphblasts?
|
large, immature lymphoid cells with high N:C (little cytoplasm) and single nucleolus
|
|
atypical lymphocytosis is the result of:
|
mononucleosis,
other viral inf's |
|
clinical presentation of hairy cell leukemia:
(3) |
1. **males** (9:1)
2. massive splenomegaly 3. pancytopenia |
|
symptoms of multiple myeloma are the result of:
|
1. mass/growth
2. secretions of malignant plasma cells (M prot, light chains) |
|
B2 microglobulin and albumin are useful for ____________ multiple myeloma
|
staging
|
|
***kapp/lambda staining is:***
|
BLUE
- blue on both = polyclonal = NOT myeloma |
|
renal dysfunction in multiple myeloma appears as:
(3) |
1. Bence Jones protein in urine
2. light chain nephropathy 3. amyloid in the glomeruli and blood vessles |
|
Bence Jones protein =
|
Ig light chain found in urine
|
|
lytic lesion =
|
destruction of an area of bone
|
|
radiculopathy =
|
nerve root compression,
as seen in AMM |
|
AL Amyloidosis is associated with:
(2) |
1. mult myeloma
2. abnormal light chains |
|
clinical presentation of AL Amyloidosis:
(2) |
1. NO classic presentation
2. kidney is most often affected (followed by liver, heart, neuro) |
|
diagnostic for AL Amyloidosis =
|
positive Congo red stain
=> apple green when polarized |
|
features of WM:
( |
1. adult disease
2. ~~ mature, monoclonal B cells |
|
classic presentation of WM =
(3) |
1. HA
2. blurred vision 3. hyperviscosity |
|
diagnostic of WM =
(2) |
SPEP/IEF showing abnormal amount of IgM;
serum viscosity |
|
marrow of WM =
|
hypercellular, with NO fat
|
|
cancer-promoting factors:
(5) |
1. obesity
2. red/processed meat 3. sat. fat 4. alcohol 5. aflatoxins |
|
cancer risk-reducing factors:
(3) |
1. fruits/veggies/beans
2. healthy body weight 3. daily exercise |
|
aflatoxin =
|
type of mycotoxin produced by Aspergillus
- commonly conatminates legumes/peanuts |