NHL

Question 1

Lymphoma RF

Answer 1

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

Infection: EBV

Question 2

NHL ix

Answer 2

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

Question 3

St Jude Staging

Answer 3

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

Question 4

LLy tx (B and T)

Answer 4

B: ALL therapy
T: ALL therapy + bortezomib

Question 5

relapsed LLy tx and px

Answer 5

HSCT in CR2
EFS <30%

Question 6

LLy px

Answer 6

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

Question 7

Burkitt lymphoma extent at dx

Answer 7

70% advanced disease at dx

25-30% abdominal
20% BM

Question 8

Burkitt Lymphoma path, IHC

Answer 8

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)

Question 9

HG NHL chemo

Answer 9

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

Question 10

HG NHL risk stratification

Answer 10

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

Question 11

HG NHL tx by risk stratification

Answer 11

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

Question 12

HG NHL relapse tx and px

Answer 12

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

OS <30%

Question 13

DLBCL clinical presentation

Answer 13

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

Question 13

Burkitt Lymphoma px

Answer 13

OS 95%
OS 85% for Stage IV

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

Question 13

DLBCL path and IHC

Answer 13

diffuse large cells with large nuclei, more cytoplasm than Burkitt

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

Question 13

DLBCL px

Answer 13

OS 95%

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

Question 14

ALCL presentation

Answer 14

70% advanced stage
75% B symptoms

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

Question 15

ALCL path and IHC

Answer 15

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

Question 16

ALCL tx response

Answer 16

MDD (minimal disseminated disease)
ALK PCR

Question 17

ALCL tx

Answer 17

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

Question 18

ANHL12P1 results

Answer 18

addition of Brentuximab increases survival
2yEFS 79%

crizotinib arm stopped 2o toxicity

Question 19

cutaneous ALCL tx

Answer 19

45% regress
solitary: resect
multifocal: chemo

Question 20

ALCL relapse tx and px

Answer 20

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

Question 21

ALCL px

Answer 21

EFS 70-85%

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

Question 22

what IHC differentiates ALCL from HL

Answer 22

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

Question 23

PTLD epi and involvement

Answer 23

1-20% of SOT 1-10% of HSCT 60-80% EBV driven LN, GI, lung, liver rare BM (15-20%)

Question 24

PTLD pathology types

Answer 24

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

Question 25

PTLD tx

Answer 25

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

Question 26

PTLD px factors

Answer 26

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

Question 27

mediastinal mass complications

Answer 27

SVCS (50%) airway compression TLS

Question 28

Primary mediastinal NHL tx

Answer 28

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

Question 29

differences from Murphy and Ann Arbor

Answer 29

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

Question 30

Burkitt lymphoma doubling time

Answer 30

12-24h

Question 31

DLBCL tx

Answer 31

resect stage I-II R-CHOP

Question 32

PMBCL epi, tx, px

Answer 32

primary mediastinal 1% peds NHL tx: DA-EPOCH-R - PET may remain + after therapy (inflammatory) 5yEFS 70-93% OS 97%

Question 33

NHL HSCT indications

Answer 33

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