NHL Flashcards
Lymphoma RF
Immune deficiencies
- Primary: Chediak Higashi, CVID, hyperIgM, IgA defic, SCID, XLA, WAS
- Acquired: HIV, post HSCT
- chromosome instability: AT, Bloom, Nijmegen, Wener
Infection: EBV
NHL ix
CBCS, TLS, LDH, ESR
CXR
CT neck to pelvis
PET
bilateral BMA/B
LP
Excisional biopsy
St Jude Staging
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
LLy tx (B and T)
B: ALL therapy
T: ALL therapy + bortezomib
relapsed LLy tx and px
HSCT in CR2
EFS <30%
LLy px
5yDFS 90%, >80% for Stage III/IV
Burkitt lymphoma extent at dx
70% advanced disease at dx
25-30% abdominal
20% BM
Burkitt Lymphoma path, IHC
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)
HG NHL chemo
COP: cyclo, VCR, pred
R-COPADM: ritux, __, doxo, MTX
R-CYM: ritux, cytarabine, MTX
CYVE: cytarabine, VCR, etop
HG NHL risk stratification
A: completely resected stage I, completed resected abdo stage II
B: all others
C: CNS or BM
HG NHL tx by risk stratification
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
HG NHL relapse tx and px
R-ICE (ifos, carbo, etop)
autoHSCT in CR2 (no evidence for allo)
OS <30%
DLBCL clinical presentation
rapidly enlarging mass
LN, liver, spleen, BM, mediastinum
Burkitt Lymphoma px
OS 95%
OS 85% for Stage IV
Poor:
- advanced stage
- LDH >2ULN
- poor risk cyto: 22q, 13q, +1q, +7q, del13q
DLBCL path and IHC
diffuse large cells with large nuclei, more cytoplasm than Burkitt
CD10, 19, 22, 42, 79a, PAX5
10-20% patchy CD30
DLBCL px
OS 95%
Poor:
- bulk (>10cm)
- M
- low BMI
- elevated serum free light chains
- BM involvement
- monoclonal serum IgM
ALCL presentation
70% advanced stage
75% B symptoms
Nodal
Extranodal: skin, liver, lung, bone, BM (10-30%)
CNS 3%
Primary cutaneous 10-20%
ALCL path and IHC
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
ALCL tx response
MDD (minimal disseminated disease)
ALK PCR
ALCL tx
prophase: dex, cyclo
Course A: dex, MTX, ifos, cytarabine, etop
Course B: dex, MTX, cyclo, doxo
Brentuximab
ANHL12P1 results
addition of Brentuximab increases survival
2yEFS 79%
crizotinib arm stopped 2o toxicity
cutaneous ALCL tx
45% regress
solitary: resect
multifocal: chemo
ALCL relapse tx and px
crizotinib –> HSCT
alloHSCT if early (<1y) relapse
EFS 50%
ALCL px
EFS 70-85%
Poor:
- mediastinum, visceral, skin, lung
- small cell or lymphohistiocytic variants
- MDD+ or MRD+
- serum anti-ALK Ab
what IHC differentiates ALCL from HL
both CD30 and 45+
CD15: ALCL neg, HL +
ALK
PTLD epi and involvement
1-20% of SOT
1-10% of HSCT
60-80% EBV driven
LN, GI, lung, liver
rare BM (15-20%)
PTLD pathology types
Nondestructive: EBV, no clonality
Polymorphic: EBV, +- clonality
Monomorphic (60-80%): EBV, clonal - resembles T/B neoplasm
PTLD tx
reduced immunosuppression
ritux
pred, low dose cyclo
monomorphic: tx per B/T neoplasm
PTLD px factors
Poor
- late (>1y)
- monomorphic (especially cHL)
- EBV neg
- multiple sites of relapse
- advanced stage
- CNS
- older age
- elevated LDH
- hypoalbuminemia
mediastinal mass complications
SVCS (50%)
airway compression
TLS
Primary mediastinal NHL tx
DA-EPOCH-R
etop, doxo, VCR, pred, ritux
differences from Murphy and Ann Arbor
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
Burkitt lymphoma doubling time
12-24h
DLBCL tx
resect stage I-II
R-CHOP
PMBCL epi, tx, px
primary mediastinal
1% peds NHL
tx: DA-EPOCH-R
- PET may remain + after therapy (inflammatory)
5yEFS 70-93% OS 97%
NHL HSCT indications
Burkitt, DLBCL, PMBCL: auto in CR2
T/B-LLy: allo in CR2