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Lymphoma > NHL: DLBCL + aggressive NHL > Flashcards

NHL: DLBCL + aggressive NHL Flashcards

1
Q

What is the most common type of aggressive NHL?

A

DLBCL

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2
Q

What is the ball park figure for survival in non treated aggressive NHL?

A

Months

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3
Q

What is the brief treatment paradigm for limited stage DLBCL?

A

R-CHOP for 3 cycles followed by
ISRT to 30-36Gy for CR
40-50Gy for PR

or

R-CHOP for 6 cycles

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4
Q

What is the brief treatment paradigm for advanced stage DLBCL?

A

R-CHOP 6-8 cycles with consideration of consolidate ISRT 30-36Gy

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5
Q

What risk factors favour treatment with consolidate RT?

A
  • bulk >7.5cm
  • skeletal involvement
  • inability to tolerate full CHT
  • residual disease after CHT on PET
  • genetic factors
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6
Q

What options are there for relapsed or refractory DLBCL?

A

Initially:
Chemoimmunotherapy followed by autologous stem cell transplant

+- RT pre or post transplant

Further relapse can be managed with Car-T cell therapy or allogenic stem cell transplant

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7
Q

Epi of aggressive NHL

A
  • 7th most common noncutaneous cancer
  • M >F (slightly)
  • 50-60% of NHL is aggressive
  • more common in low and middle income countries
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8
Q

What are the most common types of NHL (not just aggressive types)

A
  • DLBCL 30%
  • follicular 25%
  • SLL/CLL 7%
  • MZL/MALT 9%
  • mantle cell 8%
  • MZL/nodal 3%
  • primary mediastinal DLBCL 2%
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9
Q

What proportion of NHL is DLBCL?

A

30%

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10
Q

What are risk factors for (any) NHL?

A
  • older age
  • race
  • family hx
  • geographic region
  • some types of viral and bacterial infection are assoc with specific types of NHL
  • auto-immune disease
  • immune suppression (HIV, organ transplant)
  • medication (immunosuppressants, alkylating agents)
  • chemicals (hair dye, pesticides)
  • previous CLL/hairy cell leukaemia (Richter’s transformation into DLBCL in 5-10%)
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11
Q

What type of lymphoma is EBV infection a/w?

A

NK-T cell
Burkitt

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12
Q

What type of lymphoma is Hep C a/w?

A

DLBCL
Splenic MZL

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13
Q

What type of lymphoma is HTLV-1, HHV8 a/w?

A

Kaposi sarcoma
Various lymphomas in HIV+

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14
Q

What type of lymphoma is H Pylori infection a/w?

A

Gastric MALT

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15
Q

What type of lymphoma is chlamydia psittaci a/w

A

orbital MALT

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16
Q

What type of lymphoma is campylobacter jejuna a/w?

A

intestinal MALT

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17
Q

How many nodal groups are used in staging?

A

13

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18
Q

Are Waldeyer’s ring and the spleen considered nodal or extra nodal for staging?

A

extra nodal

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19
Q

Do NHL arise from cells that differentiate into T or B cells?

A

Both

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20
Q

What proportion of NHL arise from B-cell origins?

A

85-90%

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21
Q

Do NHL arise from cells originating from bone marrow or peripheral nodal tissue?

A

both

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22
Q

What “indolent” NHL is treated the same as DLBCL?

A

grade 3B follicular lymphoma

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23
Q

What is the double hit subtype of DLBCL?

A

Has a rearrangement of MYC and BCL2 or BCL6 genes.

Poor prognosis

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24
Q

What is the triple hit subtype of DLBCL?

A

Rearrangement of MYC, BCL2 and BCL6 genes.

Dismal prognosis

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25
Q

What is the classic IHC of DLBCL?

A

CD19+
CD20+
CD45+

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26
Q

What are the classic genetic alterations in DLBCL

A

t(14:18)
BCL-2, BCL-6
ALK

many others

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27
Q

What are the classic genetic alterations in primary mediastinal DLBCL?

A

No classic translocations

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28
Q

What is the IHC for primary mediastinal DLBCL?

A

CD19+
CD20+
CD5-

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29
Q

What type of patient is primary mediastinal DLBCL most common in?

A

Young female

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30
Q

What does primary mediastinal DLBCL present as?

A

Anterior mediastinal (thymic) mass

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31
Q

Is the treatment for DLBCL and primary mediastinal DLBCL the same or diff?

A

Diff

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32
Q

What are the classic genetic alterations in Mantle cell lymphoma?

A

t(11:14)
Cyclin D1

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33
Q

What is the classic IHC for mantle cell lymphoma?

A

CD19+
CD20+

CD5+ (as opposed to PM DLBCL which is CD5-)

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34
Q

What appearance microscopically is classic for Burkitt lymphoma?

A

Starry sky

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35
Q

What is the most common type of NHL in children?

A

Burkitt lymphoma

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36
Q

What is the classic genetic alteration in Burkitt lymphoma?

A

t(8:14) -> C-MYC {transcription factor}

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37
Q

What is the IHC for Burkitt lymphoma?

A

CD19+
CD20+
CD10+

CD5-

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38
Q

Why is grade 3B follicular lymphoma treated as DLBCL?

A

Genetically distinct from lower grades of FL
more aggressive

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39
Q

What is the IHC for follicular lymphoma grade 3B

A

CD19+
CD20+

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40
Q

Mantle, Burkitt, Follicular, DLCBL and PM DLBCL are what lineage of cells derived?

A

B cell

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41
Q

Anapaestic large cell, Angioimmunoblastic, Peripheral- T cell, extra nodal NK- T cell, are derived from what cell lineage?

A

T cell

42
Q

lymphoblastic lymphoma/leukaemia arise from B or T cells

A

Either

43
Q

genetics of Peripheral T cell lymphoma NOS

A

t(7:14)
t(11:14)
t (14:14)

44
Q

IHC of peripheral T cell lymphoma NOS

A

Variable T-cell
+- CD 2, 3,4, 5, 7

45
Q

Anapastic large cell lymphoma genetics

A

t (2:5) -> ALK

46
Q

Anapaestic large cell IHC

A

CD30+
EMA+

46
Q

IHC of angioimmunoblastic

A

CD4+

47
Q

genetics of angioimmunoblastic

A

no classic translocations

48
Q

genetics of extra nodal NK-T cell lymphoma, nasal type

A

loss of heterozygosity 6q

49
Q

IHC extra nodal NK T cell, nasal type

A

CD2+
CD56+

50
Q

genetics of lymphoblastic lymphoma/leukaemia

A

t(1:19)
t (9:22)

51
Q

IHC of lymphoblastic lymphoma/leukaemia

A

TdT+

52
Q

Common clinical presentation of NHL

A

Painless enlarging LN
B symptoms or other symptoms e.g fatigue, anaemia, pain, cord comp, SVCO) may be present depending on location and burden of disease

53
Q

Definition of B symptoms

A

fever >38C
drenching night sweats
weight loss >10% in 6 months

54
Q

Work up of aggressive NHL
H/P and Labs

A

H&P
*constitutional symptoms
* enlarged LN
*hepatosplenomegaly

Labs
*FBC
*LFTS, Use, electrolytes
*B2 microglobulin
*LDH
*uric acid
*Hep B serology (reactivation with ritux)
*pregnancy test

55
Q

Who should have a lumbar puncture with flow cytometry as part of their workup?

A

symptomatic
testicular
double hit
HIV associated
epidural lymphoma

56
Q

Work up of aggressive NHL
imaging

A

FDG PET standard
CT contrast

ECHO or MUGA for CHT

57
Q

What feature on PET suggests transformation in low grade lymphoma?

A

uptake >10 SUV in low grade lymphoma

58
Q

For what types of lymphoma is PET not helpful in

A

Low grade, indolent histologies e.g extranodal MZL and SLL

59
Q

What is the preferred type of biopsy for lymphoma?

A

excision biopsy for adequate pathological evaluation including morphology, nodal architecture, genomic profiling, and immunoprofiling.

Core biopsy at least if excision not possible

60
Q

What type of biopsy is insufficient for lymphoma?

A

FNA

61
Q

What is the risk of bone marrow involvement in DLBCL compared with other types of lymphoma?

A

20% risk in aggressive NHL compared with 50-80% in indolent NHL

62
Q

Is a negative PET sufficient in ruling out bone marrow involvement in DLBCL? what about in indolent NHL?

A

sufficient to rule out BM involvement in DLBCL.

Not sufficient in non aggressive NHL- bone marrow biopsy is standard

63
Q

What factors make up the IPI prognostic system?

A

LEAPS

LDH (high)
Extra nodal site (equal or more than 2)
Age (>60)
Performance status (ecog greater or equal to 2)
Stage (Ann arbor III or IV)

Each scores 1 point

64
Q

What are prognostic factors for DLBCL?

A

Age (older worse)
Bulk (>= 7.5cm)
Stage
Germinal centre subtype >non germinal centre subtype
Deauville score on post treatment PET

65
Q

Describe the 5 Deauville scores

A

Level 1: no uptake above background
Level 2: less than or equal to mediastinal blood pool
Level 3: uptake above mediastinal blood pool, less than or equal to liver
Level 4: moderately above liver
Level 5: markedly greater than liver or new lesions

66
Q

What is the natural history of DBCL/aggressive NHL

A

survival measured in months if untreated
Compared to HL, pattern of spread is less predictable and can skip nodal levels/sites

67
Q

What is 3Yr OS and PFS for IPI 0-1 in ritux era?

A

OS 91%
PFS 87%

68
Q

What is 3yr OS and PFS for IPI 2 in ritux era?

A

OS 81%
PFS 75%

69
Q

What is the 3yrs OS and PFS for IPI 3 in ritux era?

A

OS 65%
PFS59%

70
Q

What is the 3yrs OS and PFS for IPI 4-5 in ritux era?

A

OS 59%
PFS 56%

71
Q

In the Ann arbor (Lugano) staging, what dose stage I denote?

A

one node or a group of adjacent nodes

OR
single extra nodal lesion without nodal involvement (IE)

72
Q

In the Ann arbor (Lugano) staging, what dose stage II denote?

A

> = 2 nodal groups on same side of diaphragm

73
Q

In the Ann arbor (Lugano) staging, what dose stage III denote?

A

Nodes on both side of diaphragm; nodes above diaphragm with spleen involvement

74
Q

In the Ann arbor (Lugano) staging, what dose stage IV denote?

A

Additional noncontiguous extra lymphatic involvement

75
Q

In the Ann arbor (Lugano) staging, what dose A denote?

A

No systemic symptoms

2014 Lugano update doesn’t use

76
Q

In the Ann arbor (Lugano) staging, what does B denote?

A

B symptoms present

2014 Lugano update doesn’t use for NHL

77
Q

In the Ann arbor (Lugano) staging, what does E denote?

A

extra nodal involvement

2014 Lugano update doesn’t use for NHL

78
Q

In the Ann arbor (Lugano) staging, what does X denote?

A

Bulky

2014 Lugano update doesn’t use

79
Q

Is observation an appropriate treatment option for aggressive NHL

A

No,

notable exception is mantle cell with a low tumour burden

80
Q

What is the role of surgery for aggressive NHL?

A

generally limited to excision biopsy

81
Q

What is 10yr OS for IPI 0-1?

A

80%

82
Q

What is 10yr OS for IPI 2-3?

A

70%

83
Q

What is 10yr OS for IPI 4-5?

A

40%

84
Q

What is the role of CHT in treatment for aggressive NHL?

A

backbone of therapy

85
Q

What is rituximab?

A

anti-CD20 antibody
Improves 5year OS for DLBCL by approx 10% with minimal increase in toxicity

86
Q

What is R-CHOP

A

rituximab, cyclophosphamide, doxorubicin, vincristine

87
Q

What is the common treatment regime for DLBCL , favourable type?
non bulky <7.5cm, IPI 0-1

A

R-CHOP x3C + ISRT 30-36Gy

or

R-CHOP x4 for IPI 0
R-CHOP x 6 for IPI >0
then PET at 4C
if CR then consider RT
if PR then complete 6Cycles and ISRT

88
Q

What is the common treatment regime for DLBCL , activated B cell type?

A

R-CHOP x 6-8C +- RT

R-ACVBP + MTX/Leukovorin

R-CHOP + Lenalidomide

Studies suggest inferior outcomes with standard R-CHOP so some intensify CHT

89
Q

What is the common treatment regime for DLBCL double hit or triple hit

A

R- EPOCH (R-CHOP + etoposide)

R-Hyper-CVAD

Consider CNS prophylaxis
Consider consolidation with autologous Stem cell transplant

90
Q

How can CNS prophylaxis be given in high risk patients?

A

Systemic MTX
intrathecal MTX
cytarabine

91
Q

What is the common treatment regime for DLBCL transformed follicular?

A

R-CHOP x 6C +- RT

92
Q

What is the common treatment regime for Follicular lymphoma grade 3B

A

Same as DLBCL paradigm

R-CHOP +- RT

93
Q

What is the common treatment regime for primary mediastinal DLBCL?

A

R-EPOCH x 6C +-RT

R-CHOP x 6C + RT

94
Q

What chemo/treatment options are there for mantle cell?

A

R-CHOP + autologous stem cell transplant

R-Hyper-CVAD/Cytarabine/MTX

R-CHOP + RT for selected stages I-II

Bendamusitne + ritux

R-chop not curative

95
Q

What chemo options are there for Burkitt lymphoma?

A

CODOX-M

CALGB regimen

R-EPOCH

Hyper-CVAD

96
Q

What chemo options are there for extra nodal NK -T-cell nasal type?

A

SMILE +RT

DeVIC + concurrent RT

GELOX + sandwich RT

97
Q

What is the treatment paradigm for unfavourable early stage DLBCL?

A

R-CHOP x 6 + ISRT 30-36Gy

98
Q

What is early stage in DLBCL

A

stage I and II

99
Q
A

Lymphoma (2 decks)

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