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Haem/Onc > Non-Hodgkins Lymphoma, ASPHO > Flashcards

Non-Hodgkins Lymphoma, ASPHO Flashcards

1
Q

2 ways to divide up NHL?

A

T vs B cell derived, mature vs immature

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2
Q

give an immature t-cell derived NHL

A

t-lymphoblastic

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3
Q

give an immature b-cell dervied NHL

A

b-lymphoblastic

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4
Q

give a t-cell derived mature NHL

A

anaplastic large cell

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5
Q

give 3 b cell derived mature NHLs

A

burkitts, diffuse large B, primary mediastinal b cel

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6
Q

give 4 rare NHLs

A

marginal zone, follicular, peripheral T cell, NK cell, gamma delta T cell, primary CNS lymphoma, primary lymphoma of the bone

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7
Q

what is the realtive prevalence of the various NHLs?

A

Burkitts 40%…and then 20% for each: ALCL, T-LLy, DLBL

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8
Q

most NHLs (60%) are what stage at dx?

A

3-4

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9
Q

what % of NHLs are secndary maligs?

A

<1%

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10
Q

approximate realtive % of B cell lymphoblastic lymphoma and primary mediastinal b cell lymphoma within NHLs?

A

B-LLy 3%; PMBCL 1.5%

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11
Q

peds NHLs are more common with what demographcis?

A

males, younger children, caucasians

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12
Q

incidence of NHLs in people <20?

A

4 per million; 800 cases/year in US

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13
Q

Burkitts has a gender preference?

A

yes, 5x more common in males than females

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14
Q

discuss NHLs with relation to age

A

incidence increases with age, except for lymphoblastic lymphoma

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15
Q

etiology fo lymphoma?

A

EBV infection (burkitt), immunodef/immunosuppression, pesticides

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16
Q

immunodeficiences increase the risk of what?

A

NHL

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17
Q

give 5 primary immunodefs that increase risk fo NHL

A

WAS, IgA def, CVID, SCID, hyper-IgM syndrome….also chediak-higashi, x-link agammaglobuolinemia

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18
Q

give 3 acquired immunodefs that increase risk of NHL

A

following chemo for HL, immunosupprsesion after SOT, HIV

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19
Q

give 4 immunodefs from chrom instab that increase risk fo NHL

A

Ataxia telangiectasia, bloom syndrome, Nijegen breakage syndrome, werner syndrome

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20
Q

___ cell defs predipose you to ___ ___ lymphoma, often with ___ disease that is ___ ___

A

t-cell; b-cell; extra-nodal; EBV positive

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21
Q

describe the histo for burkitts

A

homogenous medium sized cells, high mitotic index, “starry sky”…KI67/MIB1>99% of cells are positive

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22
Q

burkitts: highly aggressive ___ ___ mature b cell lymphoma

A

germinal centre

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23
Q

epi for burkitts: gender, age, sporadic vs endemic?

A

males>females; 5-10 yrs, both

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24
Q

immunophenotype for burkitts? (6)

A

CD10,19,20,22,sIg+ (>90% IgM), low BCL-2 (anti-apoptotic)

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25
Q

burkitts: % that is Ki-67+

A

99%

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26
Q

3 translocations in burkitts? and 3 other changes in >50%?

A 
IgH-cMYC= t(8;14)(q24;q32)>
Igkappa-cMYC= t(2;8)(p11;q24)>
Iglamda-cMYC= t(8;22)(q24;q11)....-13q, +1q, -6q
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27
Q

burkitts is the first human tumour to what? (3)

A

be associated with a virus (EBV), be known to have recurrent translocations, associated with HIV

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28
Q

have do you treat burkitt leukemia?

A

like burkitt lymphoma

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29
Q

decribe burkitts with relation to geography (sporadic vs endemic)

A

sporadic in north america, europe; endemic in equatorial africa, brazil, turkey, new guinea

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30
Q

incidence of burkitt if sporadic vs endemic?

A

0.2 per 100k vs 10 per 100 k

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31
Q

age for burkitt if sporadic vs endemic?

A

6-12 vs 4-7

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32
Q

EBV prev in burkitts if sporadic vs endemic?

A

15% vs 95%

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33
Q

presentation for burkitts if sporadic vs endemic?

A

sporadic: abdo, nodes, marrow, cns, csf…endemic: jaw, orbit, mesentery, CNS in 1/3

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34
Q

both endemic and sporadic burkitts express what?

A

ebv latency proteins

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35
Q

do translocations in burkitts differ if sporadic vs endemic?

A

no, they’re the same

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36
Q

what makes the starry sky apperance in burkitts?

A

reactive macropahges engulfing apoptoic debris from rapidly dividing cells

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37
Q

other than in burkitts, where else can you see c-myc expression adn b-cell markers? (2)

A

DLBCL, PMBCL

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38
Q

describe histo for DLBCL

A

large cells, abundant cytoplasm

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39
Q

immunophenoytpe for DLBCL? 7

A

sIg+, CD19, 20, 22, 79a, PAX5, occasional CD30+.

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40
Q

cytogenetics for DLBCL?

A

2/3 express BCL-6; 1/3 express cMYC

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41
Q

% of Ki-67+ cells in DLBCL?

A

<90%

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42
Q

what are the subsets fo DLBCL and their %s?

A

90% germinal centre b-cell like= GCL DLBCL….10%: activated b-cell like (ABC DLBCL…t14;18…ABC more in adutls and worse prog

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43
Q

how does DLBCL present?

A

more diverse (LN, liver, spleen, marrow, mediastinum), extranodal abdo (bowel, mesentery, retroperitoneum), may present with intussusception, often adv stage

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44
Q

primary mediastinal b cell lymphoma= rare thymic b cell lymphoma…% of DLBCL?

A

20%…but overall 1% of NHL

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45
Q

PMBCL: gene profile overaps with waht?

A

classic HL

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46
Q

immunophenotype…this is for what cancer? CD19,20,22,79a,PAX5, no SIgG, CD30=80%, PD-L1, PD-L2 (70%), low MHC II

A

primary mediastinal b cell lymphoma

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47
Q

describe the genetics of PMBCL

A

9p24.1 amplifcation–> increased PD-L1, PD-L2 expression….CIITA alterations–> decreased MHCII expression…9p gains/amplification (encodes JAK2)…SOCS1 mutations = negative regulatory of JAK-STAT, IL-4 receptor activating mutations

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48
Q

how does PMBCL present?

A

SVC syndrome in 50%

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49
Q

in lymphoblastic luekemia/lymphoma, LN architecture is ___ ___

A

completely effaced…just see sheets of small round blue cells (no follicles)…get small to med sized cells with open chromatin, minute nucleoli, lymphoblasts of LL and AL are cytologically identical

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50
Q

in lymphoblastic lymphoma, get sheets fo ___ + lymphoblasts

A

Tdt+

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51
Q

what’s the difference between lymphoblastic lymphoma and leukemia?

A

lymphoma: <25% in marrow; leukemia: >25% blasts in marrow

52
Q

frequency of T lineage LL vs B?

A

75% T, 25% B

53
Q

lymphoblastic lymphoma: what % of T are stage 3-4?

A

> 90%

54
Q

lymphoblastic lymphoma: what % of B are stage 3-4?

A

10%

55
Q

sites of T lineage LL?

A

mediastimum>marrow>CNS rare

56
Q

sites of B lineage LL?

A

isolated nodes>bone, skin

57
Q

immunophenotype in T linerage LL vs B?

A

Both have Tdt

In T, see CD1a, 2, 3, 5, 7, +/-4 and 8…whereas B has CD10,19,22,79a, HLA-DR

58
Q

what types of translocations do you see in T lineage LL vs B?

A

T: TCR promoter onto trx factors…less characterized in B

59
Q

anaplastic large cell lymphoma: describe histo

A

large, pleomorphic cells, nuc can be horseshoe like “hallmark” or may be multinucleated…sometimes see perinuclear hof= perinuclear clearing

60
Q

immunophenotype: CD15 neg, CD30+, CD45+, t cell marekrs in 60%= CD3, CD43, CD45RO, TCR genes rearranged…which cancer?

A

anaplastic large cell lymphoma

61
Q

what translocation do you see in anaplastic large cell lymphoma?

A

75% have t (2;5)= NPM-ALK fusion–> overexpression of ALK…triggers PI-3K, AKT pathway

62
Q

presentation of anaplastic large cell?

A

progressive systemic sx, or highly aggressive robust inflamm state, HLH…may have cutaneous invovleent of ALK+ ALCL

63
Q

cutaenous ALCL: describe

A

CD30+, ALK neg, requires less therapy…can wax and wane….NOTE: this = different from SYSTEMIC ALCL with cutaneous INVOLVEMENT

64
Q

describe presenattion of endemic burkitts

A

jaw swelling, abdo/orbital swelling, paraspinal mass, CNS, marrow, intussusception

65
Q

describe sporadic burkitt presenation

A

rapidly expanding abdo mass/spont tumour lysis, marrow, CNS, non-specfic GI sx, intuss

66
Q

describe presentation fo DLCBL

A

more diverse (LN, liver, spleen, marrow, mediastinum), extranodal abdo (bowel, mesentery, retropetioneum), often advanced stage

67
Q

describe presentation of primary mediastinal

A

medastinal mass, lcoal invasion, rsep sx, svc sydnrome in 50%

68
Q

describe presentation fo lymphoblastic lymphoma

A

mediastinal mass, pleural/pericardial effusions, pain, dysphagia, dyspnea

69
Q

describe presentation of ALCL

A

slowly progressive, systemic sx, organs and skin, nodal, extranodal, can be very aggressive with inflammatory response adn HLH…adv disease in 2/3 but CNS and marrow are rare

70
Q

intuss occurs in which 2 lymphomas?

A

abdo burkitts, DLBCL

71
Q

onc emergeis you can see in lymphoma

A

airway compression (mediastinal mass), breathing (pulmonary eff), circ= SVC syndrome/tamponade/arrhythmia, disability = paraspinal/epidural –> pain, paralysis, electrolysis (TLS), failing organ (kidneys (ureter compression, increased uric acid), GI (obstruction, intuss, bleeding, perf, jaudncie, pancreatitis), hematological (cytopenias from marrow infiltr)

72
Q

why can you NOT intubate pts with mediastinal mass for airway protection?

A

obstruction can occur distal to ETT

73
Q

if must to steroids, radiation emergently: biopsy may become uninterpretable after how long?

A

48 hours

74
Q

how to eval NHL

A

hx, resp adn constituional sympoms (b symptoms not prog)
phys exam (LAD, signs infection, airway)
labs: cbc, inflamm markers, biochem, TSL labs
CXR
no empiric steroids….
later: CT neck and chest, CT or MRI of abdo and pelvis…most will get FDG-PET…exisional or incisional LN bx (histo, flow, cytogen, molec), bilateral BMA and bx, cerebal spinal fluid cytology

75
Q

name for staging for NHL?

A

st jude (murphy) classication

76
Q

descirbe stage 1 in murphy classification

A

1: single nodal or extranodal tumour (EXCLUDING mediastinum adn abod)

77
Q

describe stage 2 in murphy

A

a) single exranodal tumour with regional LN involvement or b) two or more nodal areas on the same side of the diaphragm, or c) two extranodal tumours on teh same side of the diphragm (with or without regional node involvement) or d) primary GI tract tumour that is resectable (without ascites…usually ileocecal with or without reginal node involvement)

78
Q

describe stage 3 in murphy

A

a) 2 extranodal tumors on opp sides of diaphram
b) 2 or more nodal areas above and below the diaphragm
c) any intrathoracic disease (lung, pleura, mediastinum, thymic)
d) all extensive primary intraabdo disease
e) all paraspinal or epidural disease

79
Q

describe stage 4 in murphy

A

BM involvement >5%, CNS involvement (other than facial nerve palsy or paraspinal disease), CSF blasts, CNS mass, CN palsy, cord comp, parameningeal disease

80
Q

what are the 2 ways to risk stratify in mature b cell lymphoma?

A

FAB/LMB and BFM

81
Q

describe FAB/LMB risk stratification for mature b cell lymphoma

A

stratum a= completely resected stage I/abdo stage II
stratum b= non-resected I-4
stratum C: CNS + or >25% blasts in marrow

82
Q

describe BFM risk strat for mature b cell lymphoma

A 
R1= completely resected stage 1 or abdo stage 2
R2= Stage 3 with LDH<500 or nonresected stage 1/2
R3= stage 3 with LDH 500-999 or stage 4 with marrow>25% blasts adn LDH<1000
R4= stage 3 with LDH at least 1000 or stage 4 wtih marrow >25% blasts and LDH at least 1000 or stage 4 with any CNS disease
83
Q

in lymphoma, who doesn’t need chemo? (2)

A

pediatric follicular (completely resected), stage 1 nodular lymph predom HL

84
Q

in NHL, who doesn’t need CNS ppx?

A

resected abdo burkitt, dlbcl

85
Q

in NHL, who does NOT have short duration/intense tx?

A

lymphblastic lymphoma

86
Q

in NHL, who DOES need rads?

A

CNS + lymphoblastic lymphoma

87
Q

structure of lymphoblastic lymphoma tx?

A

induction, consolidation, mainteance…duration fo therapy at least 2 yrs

88
Q

surival in burkitt?

A

95%

89
Q

survival in DLBCL?

A

95%

90
Q

survival in lymphoblastic lympohma?

A

92%

91
Q

survival in ALCL?

A

90%

92
Q

survival in PMBCL?

A

80%

93
Q

in NHL, higher stages–> what for tx?

A

more cycles…so based # cycles based on stage and response

94
Q

in burkitt and DLCBL what improves outcomes and when?

A

rituximab improves otucomes for high stage diseae (3 with high LDH, all stage 4)

95
Q

in pmbcl tx, includes ___ and may benefit from ___ ___

A

ritux; checkpoint inhibitor

96
Q

for ALCL, ___ monotherapy with ___, ___, or ___ can result in durable remission in relapsed ALCL

A

prolonged, brentuximab, crizotinib, vinblastine

97
Q

in T-LLy: what reduces CNS relapses of T-ALL

A

nelarabine

98
Q

in B-LLy, relapsed B-LLy has a ___ ___prognosis

A

very poor (unlike B ALL)…CD19 directed therapy may be an option

99
Q

indication for SCT in burkitt/DLCBL?

A

auto: 1st relapse (CR2)

100
Q

indication for SCT in t-lly?

A

allo: 1st relapse (CR2)

101
Q

indication for SCT in B-LLy?

A

Allo: 1st relapse CR2

102
Q

indication for SCT in PMBCL?

A

auto/all: 1st relapse, CR2…no standard of care

103
Q

indication for ST in ALCL?

A

allo: no starndard of care…maybe CR3…crizotinib, ritux, vbl monotherapy can all induce prolonged remission

104
Q

give 5 late effects of NHL therapy

A

skeletal: decreased bone grwoth/AVN due to steroids
cardiac: cardiomyoapthy, effusion, pericarditis, CHF, CAD, AMI due to anthracyclines, alkylating agents
neuro: neurocog due to IT meds, hgih dose MTX
endo: infert due to alkylating agents
malig: AML/MDS due to alkylating aents, epipodophyllotoxins…and solid tumours due to alkylating agents

105
Q

diff between HL and NHL in age?

A

HL >10, bimodal…NHL<10

106
Q

diff in spread between HL and NHL?

A

HL= continguous nodes; NHL= systemic disease

107
Q

diff in b symptoms between HL and NHL

A

HL: prog…NHL: not prog

108
Q

diff in bulk disease between HL and NHL?

A

HL: prog…NHL: not

109
Q

diff in marrow eval between HL and NHL

A

HL: yes (PET)…NHL yes (biopsy)

110
Q

diff in flow order between HL and NHL

A

HL: nO…NHL: yes

111
Q

CSF eval in HL vs NHL?

A

HL: no…NHL: yes

112
Q

CSF ppx in HL vs NHL?

A

HL: no…NHL: yes

113
Q

staging in HL vs NHL?

A

HL: Ann arbor
NHL: st jude (murphy)

114
Q

radiation in HL vs NHL?

A

YES in HL (esp slow response)…NO in NHL (soem CNS+ T-LL)

115
Q

basic diff betwen stage 1 ann arbor vs murphy?

A

ann arbor: singel node/site…mruphy: single/site but not GIT or intrathoracic

116
Q

basic diff betwen stage 2 ann arbor vs murphy?

A

ann arobor: >2 nodes/sites…mruphy: >2 nodes/sites or pirmary GI if >95% rsesected

117
Q

basic diff between stage 3 in ann arbor vs murphY?

A

AA: crosses diaphragm…M: crosses diaphragm/extensive GI involvemnt/ANY intrathoracic/paraspinal/epidural

118
Q

basic diff in stage 4 bewtwen aa and murphY?

A

aa: disseminated (>1 extarnodal site)…m: marrow or CNS

119
Q

substage in HL vs NHL

A

HL: b symptoms, bulk…non in NHL

120
Q

rituximab: target and diseases?

A

anti-CD20, burkitt, DLBCL, NLPHL

121
Q

nelarabine: mech and diseases? 2

A

purine analogue, T ALL, T-LLy

122
Q

bretuximab vedotin: mech and potential diseases?

A

anti-CD30 conjugated to MMAE…classic HL and ALCL

123
Q

crizotinib: mech and diseases?

A

ALK inhibitor; ALCL, Neuroblastoma

124
Q

nivolumab, pembrolizumab: mech adn diseases?

A

checkpoint inhbitor; reverses inactivation fo anti-tumor t cells..classic HL, NLPHL, ALCL, PMBCL

125
Q

T cell therapy: mech adn disease?

A

CTL specific for EBV LMP..EBV+ classic HL, PTLD

126
Q

bortezomib: mech and diseases?

A

proteasome inhibitor, blocks degradation of IkappaB…T-LL, T ALL, cHL

Haem/Onc (30 decks)

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