NHL Flashcards

1
Q

Lymphoma RF

A

Immune deficiencies
- Primary: Chediak Higashi, CVID, hyperIgM, IgA defic, SCID, XLA, WAS
- Acquired: HIV, post HSCT
- chromosome instability: AT, Bloom, Nijmegen, Wener

Infection: EBV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

NHL ix

A

CBCS, TLS, LDH, ESR
CXR
CT neck to pelvis
PET
bilateral BMA/B
LP
Excisional biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

St Jude Staging

A

I: single node/extranodal (excluding mediastinal or abdo)

II:
- single extranodal with regional nodes
- 2+ nodal/extranodal areas same side of diaphragm
- primary GI resectable

III:
- 2+ nodal/extranodal opposite sides of diaphragm
- intrathoracic
- primary extensive intraabdominal
- paraspinal/epidural

IV: BM, CNS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

LLy tx (B and T)

A

B: ALL therapy
T: ALL therapy + bortezomib

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

relapsed LLy tx and px

A

HSCT in CR2
EFS <30%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

LLy px

A

5yDFS 90%, >80% for Stage III/IV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Burkitt lymphoma extent at dx

A

70% advanced disease at dx

25-30% abdominal
20% BM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Burkitt Lymphoma path, IHC

A

starry sky pattern: sheets of monomorphic medium blue cells with macrophage ‘stars’

CD45, 10, 19, 20, 22, HLA-DR
MYC amplification (FISH)
- 80% t(8;14)
- 15% t(2;8)
- 5% t(8;22)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

HG NHL chemo

A

COP: cyclo, VCR, pred
R-COPADM: ritux, __, doxo, MTX
R-CYM: ritux, cytarabine, MTX
CYVE: cytarabine, VCR, etop

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

HG NHL risk stratification

A

A: completely resected stage I, completed resected abdo stage II
B: all others
C: CNS or BM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

HG NHL tx by risk stratification

A

A: COPADx2
B: COP, R-COPADM x2, R-CYM x2; transition to Group C if no CR after R-CYM 1
C: COP, R-COPADM x2, R-CYVE x2, Maintenance x2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

HG NHL relapse tx and px

A

R-ICE (ifos, carbo, etop)
autoHSCT in CR2 (no evidence for allo)

OS <30%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

DLBCL clinical presentation

A

rapidly enlarging mass
LN, liver, spleen, BM, mediastinum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Burkitt Lymphoma px

A

OS 95%
OS 85% for Stage IV

Poor:
- advanced stage
- LDH >2ULN
- poor risk cyto: 22q, 13q, +1q, +7q, del13q

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

DLBCL path and IHC

A

diffuse large cells with large nuclei, more cytoplasm than Burkitt

CD10, 19, 22, 42, 79a, PAX5
10-20% patchy CD30

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

DLBCL px

A

OS 95%

Poor:
- bulk (>10cm)
- M
- low BMI
- elevated serum free light chains
- BM involvement
- monoclonal serum IgM

14
Q

ALCL presentation

A

70% advanced stage
75% B symptoms

Nodal
Extranodal: skin, liver, lung, bone, BM (10-30%)
CNS 3%
Primary cutaneous 10-20%

15
Q

ALCL path and IHC

A

large cells with eccentric horseshoe nuclei, perinuclear hof

CD30, 45, EMA
neg CD15, TdT

ALK+ 90% systemic
- 80% t(2;5) ALK-NPM
- 15% t(1;2) ALK-TPM3

16
Q

ALCL tx response

A

MDD (minimal disseminated disease)
ALK PCR

17
Q

ALCL tx

A

prophase: dex, cyclo
Course A: dex, MTX, ifos, cytarabine, etop
Course B: dex, MTX, cyclo, doxo
Brentuximab

18
Q

ANHL12P1 results

A

addition of Brentuximab increases survival
2yEFS 79%

crizotinib arm stopped 2o toxicity

19
Q

cutaneous ALCL tx

A

45% regress
solitary: resect
multifocal: chemo

20
Q

ALCL relapse tx and px

A

crizotinib –> HSCT
alloHSCT if early (<1y) relapse
EFS 50%

21
Q

ALCL px

A

EFS 70-85%

Poor:
- mediastinum, visceral, skin, lung
- small cell or lymphohistiocytic variants
- MDD+ or MRD+
- serum anti-ALK Ab

22
Q

what IHC differentiates ALCL from HL

A

both CD30 and 45+
CD15: ALCL neg, HL +
ALK

23
Q

PTLD epi and involvement

A

1-20% of SOT
1-10% of HSCT
60-80% EBV driven

LN, GI, lung, liver
rare BM (15-20%)

24
Q

PTLD pathology types

A

Nondestructive: EBV, no clonality
Polymorphic: EBV, +- clonality
Monomorphic (60-80%): EBV, clonal - resembles T/B neoplasm

25
Q

PTLD tx

A

reduced immunosuppression
ritux
pred, low dose cyclo
monomorphic: tx per B/T neoplasm

26
Q

PTLD px factors

A

Poor
- late (>1y)
- monomorphic (especially cHL)
- EBV neg
- multiple sites of relapse
- advanced stage
- CNS
- older age
- elevated LDH
- hypoalbuminemia

27
Q

mediastinal mass complications

A

SVCS (50%)
airway compression
TLS

28
Q

Primary mediastinal NHL tx

A

DA-EPOCH-R
etop, doxo, VCR, pred, ritux

29
Q

differences from Murphy and Ann Arbor

A

Murphy: NHL
Ann Arbor: HL

Murphy
I excludes mediastinum/abdo
II includes primary GI resectable
III includes intrathoracic disease, extensive 1o intraabdominal disease, paraspinal/epidural

30
Q

Burkitt lymphoma doubling time

A

12-24h

31
Q

DLBCL tx

A

resect stage I-II
R-CHOP

32
Q

PMBCL epi, tx, px

A

primary mediastinal
1% peds NHL

tx: DA-EPOCH-R
- PET may remain + after therapy (inflammatory)

5yEFS 70-93% OS 97%

33
Q

NHL HSCT indications

A

Burkitt, DLBCL, PMBCL: auto in CR2
T/B-LLy: allo in CR2