WBC II puthoff Flashcards

1
Q

which lymphomas originate from the germinal center and are b cells

A

follicular lymphoma
burkitt lymphoma
diffuse large b cell
hodkin lymphoma

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2
Q

which MHC molecules are expressed on all nucleated cells and platelets

A

MHC class I

encoded by HLA-A,B,C

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3
Q

what virus causes adult t cell leukemia/lymphoma

A

HTLV-1

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4
Q

EBV can cause what lymphomas

A

a subset of burkitt lymphoma
3-40% of hodkin lymphomas
many b cell lymphomas, arising in the setting of T cell immunodef

rare NK neoplasms

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5
Q

what kind of LN expansion do you get in EBV infection

A

paracortical T cell

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6
Q

B cell lymphoma that presents as a malignant effusion, often in the pleural cavity assocaited with what virus

A

HHV-8

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7
Q

neoplastic proliferations of white cells of myeloid origin

A

AMLs
myelodysplastic syndromes
chronic myeloproliferative disorders

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8
Q

2/3 of NHL and virtually all hodgkin lymphomas present as

A

enlarged, non tender LNs

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9
Q

lymphocytic leukemias present with

A

bone marrow suppression

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10
Q

bone marrow aspirate shows neoplastic promyelocytes with abnormally coarse and numerous azurophilic granules
-bilobed nuclei and cell in center of field that contains multiple needle like auer rods

what is this
what is the translocation

A
APL t(15;17)
fab m3 subtupe (type of AML)
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11
Q

a child with a 10,000 platelet count has ____ until proven otherwise

A

acute lymphocytic leukemia

admit to hostpital

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12
Q

what kind of tumors induce cyclic feveres

A

B cell tumors

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13
Q

is platelet count clinically useful in hodgkin and NHL

A

no bc only has bone marrow involvement later on in the disease

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14
Q

does chronic leukemia or lymphoma have low platelet counts

A

no

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15
Q

B-ALL genotype and clinical feautres

A

t(12;21) RUNX1 and ETV6 in 25%

children, symptoms relating to marrow replacement and pancytopenia, aggressive

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16
Q

T-ALL genotype and salient clinical features

A

NOTCH1 mutations
predominanlty adolescent males
thymic masses and variable bone marrow involvment
aggressive

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17
Q

burkitt lymphoma
cell of origin
genotype
salient clinical features

A

germinal center b cell
myc, t(8;14), subset EBV associated
adolescents or young adults with extranodal masses
aggressive

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18
Q

DLBCL
COA
genotype
salient clincal features

A

germinal center or postgerm center b cell
BCL6 (30%), BCL2 (10%)
all ages but most common in older adults, often appears as a rapidly growing mass; 30% extranodal; aggressive

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19
Q

extranodal marginal zone lymphoma
COA
genotype
clinical features

A

memory b cell
t(11;18) t(1;14) t(14;18)

MALT
BCL10-IGH

arises extranodal sites in adults with chronic inflammatory diseases, indolent, may remain localized

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20
Q

follicular lymphoma
COA
genotype
clincial features

A

germinal center b cell
t(14;18) BCL2-IgH
older adults with gen lymphadenopathy and marrow involvement, indolent

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21
Q

hairy cell leukemia
COA
genotype
CF

A

memory b cell
activating BRAF mutation
older males with pancytopenia and splenomegaly; indolent

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22
Q

mantle cell lymphoma
COA
genotype
CF

A

naive b cell
t(11;14) cyclinD1-IgH
older males with disseminated disease, mod aggressive

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23
Q

CLL/SLL

COA
genotype
CF

A

naive b cell or memory b cell

trisomy 12, deletions of 11, 13, 17

older adults with bone marrow, LN, spleen, and liver disease, autoimmune hemolysis and thrombocytopenia in minority; indolent

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24
Q

adult t cell leukemia/lymphoma

COA
genotype
CF

A

helper t cell
HTLV-1 provirus present in tumor cells

adults with cutaneous lesions, marrow involement, and hypercalcemia
occurs mainly in japan , west africa, caribbean
-aggressive, fatal months to a year

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25
anaplastic large cell lymphoma COA genotype CF
cytotoxic T cell rearrangment of ALK on chromosome 2 children and young adults, usually with LN and soft tissue diseae, aggressive
26
extranodal NK/T cell lymphoma COA genotype CF
nk cell or cytoxic t cell (rare) ebv assocaited adults with destructive extranodal masses, most commonly sinonasal; aggressive
27
large granular lymphocytic leukemia COA genotype CF
cytotoxic T cell and NK cell point mutation in STAT3 adult pts with splenomegaly, neutropenia, and anemia, sometimes with autoimmune disease
28
the most frequent site of primary extranodal lymphoma in in the
GI tract and almost all are NHL | next most frequent site is the skin (cutaneous lymhoma)
29
most B-ALL in children are not really lymphomas but there is an exception
some children may manifest infiltrate in initial manifestation in ALL in certain sequestered sites like the testical
30
certain characteristic findings of T cell lymphoma
efface lymph nodes diffusely with mix of pleomorphic variable sized malignant T cells -often eosniophils and macrpohages CD2-8 bad prognosis
31
severeal hallmark cells with horseshoe like or embryoid nuclei and abundant cytopolasm
anaplastic large cell lymphoma
32
who has highest incidence of ALLs
hispanics then whites then blacks
33
immunophenotype of B-ALL
CD19, PAX5 and CD10, CD20 (more mature cells), IgM heavy chain (mature cells)
34
CD22 and TdT positive in what
ALLs
35
most common numerical or structural chromosomal changes in ALL
hyperdiploidy (only seen in B-ALL)
36
clinical features of ALL and AML
abrupt stormy onset within days to a few weeks of the first symptoms symptoms related to depression of marrow function, fatigure (anemia), fever from 2ndary infections of neutropenia and bleeding to thrombocytopenia mass effects (more common ALL) by neoplastic infiltration: bone pain from marrow expansion, gen lymphadenopathy, HSM, testicular enlargement, in T-ALL, complications to compression of large vessels and airways in mediastinum central nerous system manifestations like headache, vomiting, nerve palsies from meningeal spread more common in ALL than AML
37
favorable prognosis of ALL
``` age 2-10 low wbc count hyperdiploidy trisomies 4,7,10 t(12;21) ```
38
unfavorable prognosis ALL
under age of 2 in adolescent or adult peripheral blasts >100,000 t(9;22) BCR-ABL
39
what are the peripheral b cell neoplasms
CLL/SLL follicular lympoma diffuse large cell lymphoma burkitt lymphoma plasma cell neoplasms mantle cell lymphoma marginal zone lymphoma hairy cell leukemia
40
hypgammaglob autoimune hemolytic anemia transformation to DLBCL
CLL -increased bacterial infection
41
clincial features of hairy cell leukemia
HSM pancytopenia infections dry tap
42
SLL vs CLL
CLL with only LN inovlement with no peripheral lymphocytosis
43
CLL
normal architecture is obliterated preleuekmic bc pepole die of other things (infection) absoulte lympocyte count over 5000 most common leukemia of adults in the western world chrom translocations are rare
44
CLL/SLL immunophenotype
CD19 and 20, 23, CD5 | low level of surface Ig (IgM or IgD) is also typical
45
clinical features of CLL
- pts often asymptomatic at diagnosis - when appear they are non specific - gen lymphadenopathy and HSM in 50-60% sympt pts - leukopenia can be seen in pts with SLL and marrow involvement - excess counts of 200,000 in CLL pts with heavy tumor burdern sometimes - small monoclonal Ig spike in blood of some pts
46
asymtomatic pts with monoclonal B cells in perihperal blood but in numers too few to merit diagnosis of CLL
these abnormal b cells often have some of the same genetic aberrations seen in CLL like 13q deletion and trisomy 12, only 1% of such pts progress to symptomatic CLL per year due to other genetic lesion
47
are nuceloli seen in typical lymphocytes
no | seen in SLL
48
age group for CLL | what produced in peripheral smear
older pts | blasts in peripheral smear
49
90% ____ positivity/overexpression in follicular lymphoma translocation?
BCL2 -antagonizes apoptosis t(14;18)
50
extra sites involved in follicular lymphoma
peripheral blood involvement prodcue lymphocytosis (usually less than 20,000 cells) in 10% BM invovlement in 85% of cases and takes form of paratrabecular lymphoid aggregates splenic white pulp and hepatic portal triads freq involved white nodules on spleen
51
immunophenotype of follicular lymphoma
neoplastic cells resemble normal germinal center b cells -CD19,20,10, surface Ig, and BCL6 no CD5 expressed BCL2 in 90% of cases
52
clinical features of follicular lymphoma
painless, gen lymphadenopathy. involvement of extranodal sites, like GI tract, CNS, testis uncommon indolent waxing and waning course survival is 7-9 years and not improved by aggressive therapy -give pts low dose chemo or immunotherapy (anti-CD20 when symptomatic)
53
histologic transofmation in folliuclar lyphoma
30-50% of follicular lymphomas commonly to diffuse LBCL -less commonly burkitt lymphoma emerge assocaited with MYC
54
DLBCL often a dysregulation of what
BCL6
55
immunodeficicny associated large b cell lymphoma with what
EBV
56
primary effusion lymphoma of DLBCL assocaited with
KSH/HHV-8 in all cases - gen in HIV pts - within pleural cavity or pericardium or abdomen
57
small % of DLBCL have what mutaion | come from what tumor
t(14;18) @BCL2, lack BCL6 rearrangement | -some of these may arrise from unrecognized underlying follicular lymphomas
58
median pt age for DLBCL
60
59
clincal features DLBCL
aggressive rapidly fatal without treatment rapidly enlargin mass that can arise virtually anywhere waldeyer ring involved frequently primary or secondary involvement of liver and spleen may result in large destructive masses extranodal sites common BM involvment uncommon
60
Burkitt lymphoma types
african (endemic) occurs in face and mandible, abdominal viscera, like kidneys, ovaries, adrenals sporadic occurs in ileocecum and peritoneum In HIV pts is very aggressive
61
burkitt lymphoma features
starry sky pattern failure to express BCL2 translocations of c-MYC oncognene on chrom 8 for all uniformily pattern obliterating LN architecture, no follicles or GCs
62
essentially all endemic burkitt lymphomas are latently infected with
EBV also in 25% of hiv assocaited tumors 15-20% of sporadic cases
63
what is the MYC gene for
master txn regulator that increases expression of genes required for aerobic glycolysis, warburg effect -burkitt lymphoma is beleived to be the fastes growing human tumor
64
age and gender of burkitt
children and young adults
65
immunophenotype of burkitt
IgM, CD19,20,10, BCL6, no BCL2
66
prognosis of burkitt
very aggressive but responds well to intensive chemo most children and young adults can be cured -not as good in older adults
67
whwere does burkitt lymphoma usally arise and tumor of what cells
mature b cells that usually arise at extranodal sites