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Heme and Lymph > WBC II puthoff > Flashcards

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
Q

anaplastic large cell lymphoma
COA
genotype
CF

A

cytotoxic T cell

rearrangment of ALK on chromosome 2

children and young adults, usually with LN and soft tissue diseae, aggressive

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26
Q

extranodal NK/T cell lymphoma
COA
genotype
CF

A

nk cell or cytoxic t cell (rare)
ebv assocaited
adults with destructive extranodal masses, most commonly sinonasal; aggressive

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27
Q

large granular lymphocytic leukemia
COA
genotype
CF

A

cytotoxic T cell and NK cell
point mutation in STAT3
adult pts with splenomegaly, neutropenia, and anemia, sometimes with autoimmune disease

28
Q

the most frequent site of primary extranodal lymphoma in in the

A

GI tract and almost all are NHL

next most frequent site is the skin (cutaneous lymhoma)

29
Q

most B-ALL in children are not really lymphomas but there is an exception

A

some children may manifest infiltrate in initial manifestation in ALL in certain sequestered sites like the testical

30
Q

certain characteristic findings of T cell lymphoma

A

efface lymph nodes diffusely with mix of pleomorphic variable sized malignant T cells
-often eosniophils and macrpohages

CD2-8
bad prognosis

31
Q

severeal hallmark cells with horseshoe like or embryoid nuclei and abundant cytopolasm

A

anaplastic large cell lymphoma

32
Q

who has highest incidence of ALLs

A

hispanics then whites then blacks

33
Q

immunophenotype of B-ALL

A

CD19, PAX5 and CD10, CD20 (more mature cells), IgM heavy chain (mature cells)

34
Q

CD22 and TdT positive in what

A

ALLs

35
Q

most common numerical or structural chromosomal changes in ALL

A

hyperdiploidy (only seen in B-ALL)

36
Q

clinical features of ALL and AML

A

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
Q

favorable prognosis of ALL

A 
age 2-10
low wbc count
hyperdiploidy
trisomies 4,7,10
t(12;21)
38
Q

unfavorable prognosis ALL

A

under age of 2
in adolescent or adult
peripheral blasts >100,000

t(9;22) BCR-ABL

39
Q

what are the peripheral b cell neoplasms

A

CLL/SLL

follicular lympoma

diffuse large cell lymphoma

burkitt lymphoma

plasma cell neoplasms

mantle cell lymphoma

marginal zone lymphoma

hairy cell leukemia

40
Q

hypgammaglob
autoimune hemolytic anemia
transformation to DLBCL

A

CLL

-increased bacterial infection

41
Q

clincial features of hairy cell leukemia

A

HSM
pancytopenia
infections
dry tap

42
Q

SLL vs CLL

A

CLL with only LN inovlement with no peripheral lymphocytosis

43
Q

CLL

A

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
Q

CLL/SLL immunophenotype

A

CD19 and 20, 23, CD5

low level of surface Ig (IgM or IgD) is also typical

45
Q

clinical features of CLL

A
  • 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
Q

asymtomatic pts with monoclonal B cells in perihperal blood but in numers too few to merit diagnosis of CLL

A

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
Q

are nuceloli seen in typical lymphocytes

A

no

seen in SLL

48
Q

age group for CLL

what produced in peripheral smear

A

older pts

blasts in peripheral smear

49
Q

90% ____ positivity/overexpression in follicular lymphoma

translocation?

A

BCL2
-antagonizes apoptosis
t(14;18)

50
Q

extra sites involved in follicular lymphoma

A

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
Q

immunophenotype of follicular lymphoma

A

neoplastic cells resemble normal germinal center b cells
-CD19,20,10, surface Ig, and BCL6
no CD5 expressed
BCL2 in 90% of cases

52
Q

clinical features of follicular lymphoma

A

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
Q

histologic transofmation in folliuclar lyphoma

A

30-50% of follicular lymphomas commonly to diffuse LBCL

-less commonly burkitt lymphoma emerge assocaited with MYC

54
Q

DLBCL often a dysregulation of what

A

BCL6

55
Q

immunodeficicny associated large b cell lymphoma with what

A

EBV

56
Q

primary effusion lymphoma of DLBCL assocaited with

A

KSH/HHV-8 in all cases

  • gen in HIV pts
  • within pleural cavity or pericardium or abdomen
57
Q

small % of DLBCL have what mutaion

come from what tumor

A

t(14;18) @BCL2, lack BCL6 rearrangement

-some of these may arrise from unrecognized underlying follicular lymphomas

58
Q

median pt age for DLBCL

A

60

59
Q

clincal features DLBCL

A

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
Q

Burkitt lymphoma types

A

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
Q

burkitt lymphoma features

A

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
Q

essentially all endemic burkitt lymphomas are latently infected with

A

EBV
also in 25% of hiv assocaited tumors
15-20% of sporadic cases

63
Q

what is the MYC gene for

A

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
Q

age and gender of burkitt

A

children and young adults

65
Q

immunophenotype of burkitt

A

IgM, CD19,20,10, BCL6, no BCL2

66
Q

prognosis of burkitt

A

very aggressive but responds well to intensive chemo
most children and young adults can be cured
-not as good in older adults

67
Q

whwere does burkitt lymphoma usally arise and tumor of what cells

A

mature b cells that usually arise at extranodal sites

Heme and Lymph (4 decks)

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